The treatment of severe lymphedema is a difficult challenge. We performed lymphaticovenular anastomosis on two patients with secondary skin lesions of chronic lymphedema; one patient exhibited acquired lymphangioma circumscriptum of the vulva and the other presented elephantiasis nostras verrucosa of the lower leg. Both patients obtained a remarkable improvement in skin lesions and also in the reduction of lymphedema of the lower extremity. During a 6-month-follow-up period, constant reduction in the circumference of the lower extremities without exacerbation of skin lesions was achieved in both patients. Lymphaticovenular anastomosis is a useful surgical treatment for secondary lymphedema in the lower extremities. In addition, this surgical treatment is effective for secondary lesions of lymphedema, including acquired lymphangioma circumscriptum and elephantiasis nostras verrucosa.