Several skin conditions have been reported to occur on epidermal nevi, but it seems that co-occurrence of a vascular lesion and an epidermal nevus is a very rare phenomenon. Herein, a 17- year- old female patient who had presented with skin lesions on her thigh since 4 years ago has been reported. The lesions were two oval plaques consisted of multiple brown- tinted, hyperkereratotic papules that surrounded several dark red papules. One of the lesions was totally excised and the specimen was sent for histopathologic evalution. Routine light microscopic examination of the slides revealed histopathologic features of both epidermal nevus and angiokeratoma. The clinical presentation of this case suggested the co- occurrence of an epidermal nevus and angiokeratoma, which was confirmed after histopathological study.