Abstract:Agenesis of the dorsal pancreas (ADP) is an extremely rare congenital pancreatic malformation characterized by the absence or hypoplasia of the pancreatic body and tail. Its pathogenesis is closely related to abnormal embryonic development of the ventral and dorsal pancreatic buds, governed by a complex network of transcription factors, including HLXB9, HNF1B, PDX1, PTF1A, GATA4, and GATA6. The clinical spectrum of ADP is highly variable, ranging from asymptomatic cases to manifestations such as abdominal pain, diabetes mellitus, or pancreatitis. Imaging modalities-including ultrasonography, CT, magnetic resonance cholangiopancreatography, and endoscopic retrograde cholangio-pancreatography-serve as the main diagnostic tools, with characteristic findings of absent pancreatic body and tail accompanied by compensatory enlargement of the pancreatic head. ADP is frequently associated with congenital anomalies of the kidney, biliary tract, cardiovascular system, or genital organs. Management is primarily symptomatic, with insulin replacement for diabetes and pancreatic enzyme supplementation for exocrine insufficiency. Advances in genetic sequencing and stem cell research have deepened understanding of the pathogenesis, genetic background, and potential therapeutic strategies of ADP. This review summarizes current progress in embryology, genetics, clinical features, diagnosis, and treatment of ADP, aiming to improve clinical recognition and guide future investigations.