Two cases of postmastectomy lymphangiosarcoma seen at our department are reported. One case, treated by amputation, died 1 year 4 months later and the other case, treated by local excision followed by amputation, died 7 months later. Twenty cases from the Japanese literature, including our 2 cases, are reviewed herein. The initial treatment for the other 18 cases was as follows: amputation in 8 cases, local excision in 2, radiotherapy in 4 and chemotherapy in 4. Fifteen of 16 cases eligible for follow up were dead within 20 months after treatment. Although the knowledge available as to the results of the treatment of postmastectomy lymphangiosarcoma is still insufficient to indicate the optimal therapeutic approach, early recognition of the lesion and prompt radical ablative surgery seem to offer the best chance for survival.