Sixteen-year-old mentally retarded mate patient presented with a giant malignant peripheral nerve sheath tumor (MPNST) on his neck. Surgery was the chosen method of treatment by the help of a neurosurgeon for dissecting the mass from the nerves of the brachial plexus and a thorasic surgeon for removing the mass from the superior mediastinum. A mass of 2 kg in weight was excised gross totally. He had an early recurrence in the second month of the follow-up period despite postoperative irradiation therapy. He died of his disease due to the compression of vena cava superior. (C) 2004 Elsevier Ireland Ltd. All rights reserved.