Angiokeratoma of Fordyce is a relatively common type of angiokeratoma that is characterized by small, multiple, purplish to dark red papules on the scrotum. Pathogenesis is unknown, but it has been suggested that increased venous pressure caused by varicocele, hernia or tumor of the epididymis can induce angiokeratoma of Fordyce. We report a case of unilateral angiokeratoma of Fordyce with ... [Show full abstract] varicocele in a 30 year-old man. He presented with approximatly 2 mm sized, purplish to dark red, and multiple hyperkeratotic papules on the left scrotum. The lesions were strictly limited to the left side of the scrotum and associated with varicocele on the same side. Histopathologically, hyperkeratosis, acanthosis with elongation of rete ridges and dilated papillary vessels are presented. Based on this clinical and histologic finding, we diagnosed this case as unilateral angiokeratoma of Fordyce with varicocele. The patient was treated with varicocelectomy for left varicocele and then cryotherapy for skin lesions.