A case of Gougerot-Hailey-Hailey benign familial pemphigus is reported. This genodermatosis inherited in an autosomal dominant pattern is shown to occur more often in men aged 20-30 years and older. The authors provide brief information on etiology and pathogenesis of this disease, variants of its clinical presentation, and features of differential diagnosis. It is emphasized that histology and family history of the disease play the crucial role in proper diagnosis. A clinical case of a 34-year-old female patient M., who has been observed by dermatologists for 8 years with different diagnoses (candidosis, eczema, dermatitis), is reported. Given the nature of the disease, seasonal variation of the rash (during summer season), family history (the mother had similar disease for over 20 years), as well as the characteristic histological changes (acantholysis accompanied by formation of suprabasal bubbles and gaps, papillary excrescences protruding into these cavities, and dyskeratotic foci with the formation of "round bodies" in the granular layer), the patient was diagnosed with Gougerot-Hailey-Hailey chronic benign family pemphigus.