The term “angiokeratoma” is applied to several distinct conditions with vascular skin lesions, the histology of which shows superficial vascular ectasia and overlying hyperkeratosis.1 The angiokeratomas should be regarded as capillary vascular malformation rather than hemangiomas.1 Apart from angiokeratoma corporis diffusum (Fabry), which is a disorder of phospholipid metabolism, at least four types of purely cutaneous angiokeratoma are known: the Mibelli type, the Fordyce type, angiokeratoma circumscriptum and “solitary and multiple angiokeratoma” (Imperial and Helwig). We present here a case of angiokeratoma circumscriptum and a brief review of the literature.