Porokeratosis is a rare disease with an autosomal dominant type of inheritance. It is customary to distinguish classic Mibelli porokeratosis, as well as disseminated and localized variations of the course of dermatosis, which differ in genetic predisposition, trigger factors, and treatment approaches. The article describes the variants of the disease – eruptive itchy papular malformation that occurs on the extremities against the background of microcirculation disorders and giant, developed on the skin of the trunk. The main methods of treatment of porokeratosis are also described.