Plexiform schwannoma is a benign tumor originated in the sheath of the peripheral nerves that is composed of Schwann cells that are arranged in palisade. These lesions usually arise from the skin being less frequent in intraabdominal organs. We describe, according to the reviewed literature, the second case of plexiform schwannoma arising from the duodenum. A 49 year old patient with a history of vomiting, diffuse abdominal discomfort and weight loss. The complementary tests were inconclusive. The patient was operated evidencing in the operation room a stenosis of the third duodenal portion without any other alterations. The pathological anatomy reported a submucosal lesion formed by multiple nodules composed by a proliferation of spindle cells that were positive for S-100.