A 24-year-old married woman had an angiokeratoma of the vulva measuring 3 x 3 x 4 mm localized at the clitoris. The nodule was noticed when she was pregnant with her first child. This tumor grew with occasional tenderness and bleeding during pregnancy, but its size did not change after delivery. The growth of this tumor might be attributable to an increase in venous pressure or serum progesterone levels; therefore this disorder might be classified as a subtype of angiokeratoma of the vulva. This is the first case of angiokeratoma localized at the clitoris.