Undifferentiated pleomorphic sarcoma (UPS) was earlier termed as malignant fibrous histiocytoma (MFH). UPH mainly affects extremities and retroperitoneum with the age predisposition of 50+. We present a case of 16 year old boy with malignant UPS; primary in thigh with lung metastases showing an excellent response to multidisciplinary approach involving surgeryfollowed by systemic combination chemotherapy. The treatment of the patient with metastatic UPS/MFH was successfulutilizing a multidisciplinary approach; although, this type of sarcoma carries a poor prognosis and seen to be insensitive to both chemotherapy and radiotherapy.

Keywords : Undifferentiated pleomorphic sarcoma, Malignant fibrous histiocytoma, Chemo radiotherapy.