Angiokeratoma circumscriptum is one of five types in the group of the angiokeratomas. These lesions are usually present at birth and are not related with systemic diseases. Angiokeratoma circumscriptums are hyperkeratotic papules or nodules occurring frequently unilaterally on the leg. These lesions are very rare and very few cases have been reported since its first description. It is of particular clinical importance because of its morphologic similarities to malignant skin tumors such as malignant melanoma or pigmented basal cell carcinoma. Therefore, this rare but significant lesion must be recognized by physician and surgeons who manage skin tumors.