Congenital hypertrophy of the retinal pigment epithelium is a benign pigmented formation on the globe fundus, which has a characteristic appearance during ophthalmoscopy and is not prone to significant growth as well as malignancy. Nevertheless, one of the forms of congenital hypertrophy of the retinal pigment epithelium is associated with familial adenomatous polyposis, therefore, for timely follow-up examination of patients, it is necessary to know the differential diagnostic criteria of various forms of this disease. The article presents three clinical cases and as well as summarizes information about various forms of congenital hypertrophy of the retinal pigment epithelium.