Melanoma-associated vitelliform retinopathy is a manifestation of paraneoplastic syndrome in skin melanoma. Paraneoplastic syndrome, while not being a tumor or a metastatic disease, is regarded as a tumor-associated disease related to extraocular localization of neoplasm. In this clinical case, the diagnosis of melanoma-associated vitelliform retinopathy was based on a combination of clinical, angiographic, autofluorescence and morphometric signs of bilateral lesion. Analysis of the case showed that in common oncological diseases and complaints of visual impairment, examination of eye fundus is mandatory in order to timely diagnose the changes associated with tumor lesion. Detection of bilateral lesions with oval grey-yellow multiple foci at the level of retinal pigment epithelium may indicate melanoma-associated vitelliform retinopathy that requires diagnostic search for skin melanoma. A complex of instrumental studies including fluorescent angiography, optical coherence tomography and autofluorescence with feature identification allowed establishing the correct diagnosis in the particular case.