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The Meckel’s divetriculum has a prevalence of the 0.3-3%. It is surgically removed only when arise a complication or a neoplasia. The Meckel’s diverticulum tumors are unfrequent and observed in only the 0.5-3.2% of the Meckel’s 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 Meckel’s diverticulum.