A case of a rare comedogenic nevus is presented. The incidence and prevalence of the disease have not been determined. Usually a nevus is congenital or manifests itself in early childhood. The nevus is formed as a result of inadequate development of the mesodermal part of the sebaceous hair follicle and insufficient differentiation of its epidermal part. As a result, the follicle is unable to form full-fledged hair but produces soft keratin. The nevus is asymptomatic and is represented by expanded hair follicles filled with soft horny masses. In most cases, the nevus is small but can reach gigantic proportions occupying half of the body; multiple comedonic nevi have been described. During puberty it is possible to add an inflammatory component with cysts and fistulas. Differential diagnosis is carried out with a linear nevus, Favre’s disease — Rakusho, follicular keratosis. To confirm the diagnosis a histological examination is necessary where rudimentary hair follicles filled with layered horny masses are found. When a comedonic nevus is combined with a pathology of the skeleton, skin, eyes, and the central nervous system, one speaks of comedonic nevus syndrome. Treatment in most cases is symptomatic (topical keratolytic agents, moisturizers), possibly topical aromatic retinoids. Systemic aromatic retinoids are rarely prescribed due to the need for their long-term use and the possible side effects. The surgical removal of nevus with subsequent plastic surgery of the skin are descripted in the literature.