Results: The lesion was excised and the histological examination revealed a tumour involving lamina propria, composed of large dilated vascular spaces, lined by normal appearing endothelium and filled with blood or with fibrin thrombi. The overlying epithelium showed variable degree of acanthosis and hyperkeratosis. Accordingly the diagnosis of angiokeratoma was made. On clinico-laboratory examination no angiokeratomas were found anywhere on the skin as well as no other malformation or metabolic disorder. The patient received no further treatment and 2 years later remains disease free.