Angiokeratoma of Fordyce (scrotum) is typically small, multiple, red to purplish papules located on the scrotum, shaft of penis or labia majora. It is recognized as an acquired papillary dermal dilatation of capillaries arising in middle age or later, but its exact pathogenesis including origin of vessel type is uncertain. We report 4 cases of angiokeratoma of Fordyce those can be considered as an acquired dilatation of lymphatic vessel, by performing an immunohistochemical study using lymphatic markers such as D2-40 and Prox-1. The patient 1, 69-year-old male (10-year history), presented with multiple vesicles filled with red and purple fluid. The patient 2, 80-year-old male (3-year), presented with multiple purplish papules. The patient 3, 49-year-old male (4-year), presented with multiple pinkish keratotic papules. The patient 4, 35-year-old male (8-year), presented with multiple reddish papules. The skin lesions were located on the scrotum and/or penile shaft. Histopathology showed diagnostic findings of angiokeratoma in all specimens. And the dilated vessels revealed focally positive membrane staining for D2-40 in 2 cases and positive nuclear staining for Prox-1 in all cases. Through these 4 cases, we should consider about lymphatic derivation of angiokeratoma of Fordyce that demonstrated by expression lymphatic markers D2-40 and especially Prox-1, and about the possibility that it is a kind of lymphangiectasia.