INTRODUCTIONParagangliomas are relatively rare endocrine tumours that arise from paraganglionic tissue, a widely dispersed collection of specialized neural crest cells. In the absence of histological diagnosis and symptoms of catecholamine excess, paragangliomas may be mistaken for GISTs. Approximately, 50 cases of non-functional retroperitoneal paragangliomas were reported in the literature. We report a case of paraganglioma of the duodenum, which emphasizes the necessity to include extra-adrenal paraganglioma in the differential diagnosis and management of retroperitoneal tumors.