The plexosarcomas or gastrointestinal autonomic nerve tumors (GAN tumors) are very rare neoplasms originating from the gastrointestinal autonomic nervous system. They have been differentiated from the other gastrointestinal stromal tumors due to ultrastructural features showed by electronic microscopy. Their mesenteric localization rare than the gastric and intestinal localization, and it gives a very poor and specific clinical symptomatology. It's very difficult to make a correct diagnosis. The most important prognostic factors influencing the survival seem to be the tumor size and the mitotic activity. The authors describe a case of multiple Plexosarcomas occurred in a young patient one year after a surgical intervention for ileal resection to remove a benign schwannoma. This patient wasn't a carrier of multiple Neurofibromatosis. The diagnostic difficulties before surgical operation are stressed and the importance of surgery as the only diagnostic and therapeutic choice is underlined, even if local recurrences occurred in more than 50% of the treated cases.