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Angiokeratomas are a group of vascular ectasias that involve the papillary dermis and may produce papillomatosis, acanthosis and hyperkeratosis of the epidermis. Angiokeratoma circumscriptum is the least common variant among many types. Angiokeratoma circumscriptum neviforme is a still rarer variety of angiokeratoma, which is classically seen at birth. We report here a case of congenital, linear, unilateral, verrucous plaques on the leg of a young girl, diagnosed as angiokeratoma circumscriptum neviforme (ACN).