Schwannomas are benign tumors derived from glial cells of peripheral nerve sheaths. They usually occur in association with Von Recklinghausen's disease and most commonly are observed on the head, neck, and upper extremities. Rare morphological variants may be misinterpreted as a malignancy under microscopic examination. The study presented a case of a sporadic "ancient" Schwannoma cell tumor located in the retroperitoneum. Literature data concerning diagnosis and treatment of the above-mentioned pathology was also reviewed.