Malignant schwannoma is a malignant neoplasm of nerve sheath cells. It develops in a solitary fashion or associated with Von Recklinghausen disease.

We experienced a malignant schwannoma in a 20-year old man with Von Recklinghausen disease. The lesion was gure, irregular, cystic and solid mass with septations in the right retroperitoneal region, inferior to the kidney. The histologic findings of retroperitoneal and pulmonary mass was compatible with malignant schwannoma. Incomplete excision due to infiltration to the adjacent tissue, radiotherapy and combined chemotherapy were performed.

We report a case of retroperitoneal malignant schwannoma associated with Von recklinghausen disease.