Hemangiopericytoma (HPC) is a rare soft tissue tumor The few published reports account for the little information available on its clinical management. Here the authors report the successful treatment of an adolescent girl with rare HPC of the tongue. After incomplete surgical excision of the tumor she was admitted to the Hematology-Oncology Department and was treated with a 3-drug combination regimen (ifosfamide, actinomycin D, vincristine) for 8 weeks. She achieved partial remission in week 9 based on the magnetic resonance imaging (MRI)findings. Conventional radiation therapy was initiated at week 9 and continued until week 16. At week 20, according to the MRI findings, she achieved complete remission and continuation therapy was initiated. The young girl has been alive without evidence of the disease for the last 3 years of follow-up. In conclusion, the current report indicates that in cases of incomplete surgical excision of the tumor, chemotherapy and radiotherapy seem to be effective.