ABSTRACTA 21‐year‐old man with angiokeratoma corporis circumscriptum neviforme (ACCN) involving his left buttock, thigh, leg, and foot was treated by resection of the lesions followed by skingrafts. The lesions had gradually spread and proliferated for 10 years following previous surgical therapy in our department.Histopathologic examination of the lesions revealed a marked hyperkeratosis of the epidermis; the upper dermis showed capillary dilatation and proliferation, and a distinct proliferation of immature endothelial cells of capillaries was noted in the middle and deep dermis.Additionally, 34 cases of ACCN reported in Japan are reviewed. Possible etiologic factors considered are a lack of oxygen and nutrition in the tissue due to a disturbance of blood circulation, and repeated trauma to the lesi