Ectopic pancreas refers to pancreatic tissue that lacks vascular or anatomical connection with the main body of the pancreas. Superior mesenteric artery syndrome involves the compression of the third part of the duodenum between the aorta and the superior mesenteric artery. We present a case of a 12-year-old female with a 4-year history of recurrent episodes of severe epigastric pain, improved by sitting and leaning forward, and occurred monthly for nearly 48 h. Radiological findings confirmed the diagnosis of superior mesenteric artery syndrome. However, further investigation revealed the presence of ectopic pancreas. Surgical intervention successfully treated both conditions. The patient presented with severe abdominal pain, and although there was a diagnosis of superior mesenteric artery syndrome, the pain was not entirely attributable to this condition, as there was no correlation with food intake, and the degree of obstruction was mild. Further investigations revealed the presence of ectopic pancreas, which provided an explanation for the severity of the pain. This case highlights the need for further research on the potential association between superior mesenteric artery syndrome and ectopic pancreas.