Congenital infantile fibrosarcoma is a rare soft tissue neoplasm most commonly involving the extremities and are usually present at birth. These tumors are notoriously misdiagnosed at birth as either hemangiomas or lymphatic malformations. Although histologically similar to fibrosarcomas occurring in adults, the congenital lesions differ in their clinical behavior; metastases are rare, local recurrence is common, and prognosis is good with wide local excision combined with chemotherapy. We herewith report such a rare case with review of the recent literatures.