Malignant heamangioendothelioma (MHE) of the scalp is a highly malignant neoplasm with few long term survivors. Because of its rarity and high mortality rate, little is known about effective treatment for disseminated MHE. Although IL-2 immunotherapy have been enthusiastically studied in Japan, its clinical effectivenss of preventing the metastasis is questionable. We report a patient with metastatic MHE of the scalp who achieved a long-term clinical remission after treatment with cisplatinum and ifosfamide. The 12-year course of his disease is the longest reported survival in Japan with documented metastatic MHE of the scalp. As several patients have been effective by general chemotherapy in the literature, some new chemotherapeutic agents should be undertaken to MHE patients in the future.