Nevi are congenital formations that appear on the skin from birth or in early childhood, are very common in healthy children and, as a rule, are harmless. The article deals with epidermal nevi formed from epidermal cells and skin appendages (sebaceous and sweat glands, hair follicles). Particular attention is paid to the syndromes of epidermal nevi, which are characterized by a combination of skin rashes with systemic manifestations. Correct diagnosis of different subtypes of nevi, their differential diagnosis with other pigment formations (including melanomas) and non- melanoma skin cancer, as well as the recognition of non-uniform syndromes will help to determine the pediatrician correct tactics of management of patients, further counseling and assess the prognosis of the disease. Early diagnosis using dermatoscopy and modern techniques based on artificial intelligence is most significant in children before the development of progressive symptoms or neurological disorders. In the detection of epidermal nevus syndromes, consultations of related specialists (neurologists, traumatologists, cardiologists, etc.) are recommended. © 2020, Pediatria Ltd.. All rights reserved.