Introduction:Agenesis of the dorsal pancreas (ADP) is a clinically rare entity that occasionally presents with abdominal pain. It is also association with various disorders of glucose metabolism.Case presentation:A 23-year-old male presented with continuous epigastric pain for 4 h and intermittent vomiting. He has a history of experiencing recurrent abdominal pain and diarrhoea for the past 5 years. Additionally, he has been diagnosed with type 1 diabetes mellitus for 15 years. Contrast-enhanced computed tomography of the abdomen showed the absence of body and tail of the pancreas.Discussion:ADP is caused by unknown factors, but may be linked to genetic mutations or changes in signalling pathways related to retinoic acid and hedgehog. Symptoms can be absent, but may include abdominal pain, pancreatitis, and hyperglycaemia due to beta-cell dysfunction and insulin deficiency. Imaging modalities, such as contrast tomography or magnetic resonance cholangiopancreatography, or endoscopic retrograde cholangiopancreatography, are crucial in the diagnosis of ADP.Conclusion:It is important to consider ADP as a differential diagnosis in patients with glucose metabolism disorders and associated symptoms such as abdominal pain, pancreatitis, or steatorrhea. It requires the combined use of imaging modalities such as ultrasound, contrast tomography, magnetic resonance cholangiopancreatography, or endoscopic retrograde cholangiopancreatography, as ultrasound alone may not provide a complete diagnosis.