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İstanbul Üniversitesi Onkoloji Enstitüsü, Radyasyon Onkolojisi AD., İstanbul Maffucci's Syndrome is defined as the association of multiple encondromas and hemangiomas. Maffucci's Syndrome combined with vascular endothelial tumor is a rare situation and there are a few literature as case reports. The patient, a 18 year old man, presented with Maffucci's Syndrome and hemangioendotheliomas to Istanbul University Institute of Oncology on December 1998. In this case report, Maffucci's Syndrome which is locally aggressive with no metastatic capacity is analyzed and our treatment approach together with the literature documentation is discussed. Keywords :