OBJECTIVES To report a new case of giant scrotal lymphedema due to Milroy's disease, its treatment and outcome. METHODS A 27-year-old man with generalized congenital lymphedema presented with a giant scrotal mass which interfered with his daily activities and physiological necessities. Physical examination showed a scrotal mass 40 x 40 cm in size and a normal penis. CT scan showed a homogeneous mass, thickened vaginal tunica, and bilateral hydrocele. RESULTS A surgical procedure was performed including mass resection (5.6 kg), and bilateral hydrocelectomy. Skin defect was covered with skin grafts. CONCLUSIONS Several therapeutic alternatives have been suggested for Milroy's disease with genital involvement. Nevertheless, when complications are as severe as in the present case, the only valid therapy is surgery.