The Meckels divetriculum has a prevalence of the 0.3-3%. It is surgically removed only when arise a complication or a neoplasia. The Meckels diverticulum tumors are unfrequent and observed in only the 0.5-3.2% of the Meckels diverticula. The 12% of these tumors are a GIST. We report a case of a gastrointestinal stromal tumor with neural immunophenotype, observed in a patient presenting a deep venous thrombosis perhaps with a paraneoplastic origin. We also report some observations about the state of the art about the GIST management and particularly of all of them arising in a Meckels diverticulum.