Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma of late adult file. It is extremely rare in children, though, and its existence in the pediatric population remains controversial. Although the most common site of tumor in children is the extremities, which is similar to findings of adults' series, different sites have been reported in children. Because of the rarity of this tumor in childhood, the approach to treatment of MFH is based primarily on the experience with adults. We present the clinical and pathologic features of an eight-year-old boy with MFH located on his left retroperitoneum and also review the literature.