A rare clinical case of nevus of Ota in a 6-year-old child is described. The clinical picture and diagnostic methods used in this case are reflected. On the example of the patients presented in this article, differential diagnosis with alkaptonuria is fully described. Given the risk of transition to skin melanoma and ocular melanoma, patients with nevus of Ota should be observed by Dermatologist and Ophthalmologist annually. Such patients should also strictly avoid exposition to any ultraviolet radiation and exclude possible risk factors traumatizing the nevus. Key words: nevus, Ota nevus, alkaptonuria, skin melanoma, eye melanoma.