Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 9 2 2024 11 06 79 84 Ali Goudarzi Radiology department, Shiraz University of Medical Sciences, Shiraz, Iran. AND Student Research Commitee, Shiraz University of Medical Sciences, Shiraz, Iran. Leila Ostovar Diagnostic Radiologist, Rafsanjan University of Medical Sciences, Rafsanjan, Iran. 2024 09 18 2024 10 01 Agenesis of the dorsal pancreas is a rare congenital anomaly that often remains asymptomatic and is  requently discovered incidentally during imaging for other conditions. Its association with Müllerian anomalies, such as uterus didelphys, suggests that both conditions may stem from similar embryological disruptions  during early fetal development. This report aims to raise awareness of Agenesis of the Dorsal Pancreas (ADP) and its potential associations with Müllerian anomalies. By presenting a detailed case study, we seek to address the diagnostic challenges faced by healthcare professionals and the implications for patient management, emphasizing the importance of early detection and appropriate intervention. We present a case involving a 24-year-old Iranian female who presented with a 10-day history of abdominal pain and vomiting. Initial ultrasound imaging revealed moderate hydroureteronephrosis in the right kidney, prompting further investigation. A contrast-enhanced CT scan was performed, which not only confirmed ureteropelvic junction obstruction but also unexpectedly revealed dorsal pancreatic agenesis. In addition to these findings, the patient was diagnosed with uterus didelphys, highlighting a significant developmental anomaly. This case underscores the necessity for increased awareness of ADP and its associations with Müllerian anomalies among healthcare providers. Early and accurate diagnosis through advanced imaging modalities significantly improves patient outcomes and facilitates better management strategies for associated complications. https://crcp.tums.ac.ir/index.php/crcp/article/view/987 https://crcp.tums.ac.ir/index.php/crcp/article/download/987/661