Aberrant pancreas of the duodenal wall (APD) and duodenal diaphragm (DD) are two rare entities, which developed during duodenal embryogenesis. Occurrence, diagnosis and therapeutic approach of these lesions are different. Herein, we report the first case of this exceptional association in a man who had no symptoms. A surgical resection of both lesions was performed and the outcome was uneventful. Embryogenesis, morphological characteristics and treatment are discussed.