Pancreatic schwannoma is a very uncommon tumor of the pancreas, with only 27 cases reported. Most pancreatic schwannomas are benign, with only four malignant tumors reported. We describe a case of giant malignant schwannoma of the pancreatic body and tail, which involved the transverse colon. The tumor was treated successfully with en bloc distal splenopancreatectomy and colon resection. This is believed to be the first reported radical operation for malignant schwannoma of the pancreatic body, with infiltration of the transverse colon, with excellent long-term results. The patient is alive and well 28 mo after the operation. The authors conclude that pancreatic schwannomas should be considered in the differential diagnosis of cystic neoplasms of the pancreas, although the diagnosis can only be confirmed by microscopic examination. In the case of the benign tumors, local excision is adequate, but in the case of malignant schwannoma, oncological standards must be fulfilled.