A review of published data as well as a clinical case of nevoid hyperkeratosis in a young girl are presented. Nevoid hyperkeratosis of the nipple-areola complex is a rare benign dermatosis with favorable course, mainly occurring in young women. Most publications describe series of clinical cases. Etiopathogenesis of the disease is not clarified, endocrine disorders are considered to be one of the possible causes. No in-depth genetic studies have been performed to date. Existing treatment approaches include short course of retinoids administration, topical application of keratolytic and corticosteroid ointments, retinoic acid drugs and vitamin D derivatives (calcipotriol, tacalcitol), therapy with carbon dioxide laser, cryodestruction. This report describes the disease’s clinical manifestations in the patient, dermatoscopic and pathohistological skin pattern in the lesion foci. A positive effect of the comprehensive treatment, which included administered vitamin A, application of 2—5% salicylic acid ointment and 0.1% retinoic acid ointment, on the disease’s clinical manifestations has been shown. Review of the literature data shows a rare incidence of nevoid hyperkeratosis of the nipple-areola complex. The obtained results of pathohistological examination of the affected patient’s skin and positive dynamics of the clinical picture in response to the therapy confirm the opinion of other authors about the benignity of this disease.