Intracellular copper deposits impede inhibitor of apoptosis proteins, which eventually causes apoptotic cell death. The clinical presentation varies from predominantly hepatic to predominantly neurologic and shows great heterogeneity regarding severity, age of onset and initial symptoms. Wilson’s GSI-IX disease results in severe disability and death if untreated. The key neurological features comprise extrapyramidal symptoms, ataxia, dystonia, seizures and psychiatric symptoms, such as personality changes, depression and psychosis. Structural changes in the brain of Wilson’s disease patients have been well documented by magnetic resonance imaging, which has revealed lesions of the basal ganglia, midbrain, pons and cerebellum and widespread cortical atrophy and white matter changes. Histological studies have reported necrosis, gliosis and cystic changes in the brainstem, thalamus, cerebellum and cerebral cortex of Wilson’s disease patients. The functional consequences of these structural changes have been demonstrated in the acoustic, sensory, motor and visual systems and are reflected by disordered multimodality evoked potentials. Visual evoked potentials have been reported to be abnormal in approximately 50% of symptomatic Wilson’s disease patients. Common ocular findings of Wilson’s disease include the Kayser–Fleischer ring and sunflower cataracts. Both are due to copper deposition and do not cause visual impairment, suggesting that the observed pathologies in VEPs may be explained by retroocular changes. However, altered flash electroretinograms in Wilson’s disease are indicative of a retinal pathology. Optical coherence tomography is a fast and non-invasive technique and the latest generation of OCT devices is capable of depicting retinal changes at nearly the cellular level. In this study, we used up-to-date OCT technology to analyze the retinal changes in Wilson’s disease patients. We compared the morphological changes measured by a state-of-theart spectral domain OCT device with VEPs as functional parameters and correlated these findings with laboratory parameters and a clinical Wilson’s disease score. We were able to reproduce previously reported findings that indicate that in Wilson’s disease, VEP latencies are delayed. We believe that the prolonged P100 latencies are likely to reflect a slowed conduction velocity of the visual tract caused by copper deposits. A structural analysis of the retina by OCT revealed reduced thickness of the RNFL, total macula and GCIP, clearly indicating pathological changes to the retinal ganglion cells and their axons in the retina. In line with previous publications, the VEP amplitudes of Wilson’s disease patients were unchanged compared with controls. However, in Wilson’s disease patients, low VEP amplitudes tended to be associated with thinner RNFL, GCIP and total macular thickness, although these correlations failed to reach significance. In other diseases such as multiple sclerosis, the VEP amplitude is reported to correlate with the RNFL thickness. It is possible that the extent of axonal loss in Wilson’s disease patients was not sufficient to significantly reduce the VEP amplitude. However, these findings indicate that OCT may be a more sensitive parameter of axonal loss in Wilson’s disease than VEP amplitudes. To our knowledge, no histopathological studies analyzing the retinae of patients with Wilson’s disease have been reported, so the exact mechanisms of retinal degeneration in these patients remain unclear. However, the reduction of RNFL thickness in Wilson’s disease reflects degeneration of the retinal ganglion cell axons and degeneration of the retinal ganglion cells themselves and is likely to account for the observed reduced thickness of the GCIP complex.