Background. Superior mesenteric artery syndrome (SMAS) occurs when the duodenum is compressed between the two arteries, superior mesenteric artery and aorta. The complications of this rarely found disorder in children range from causing trouble in duodenal functions to intestinal obstruction which is potentially life-threatening. Case Presentation. Here we are reporting a case of SMAS in a 6-year-old girl with the complaint of chronic abdominal pain since 3 years. She suffered from growth failure, while different workups were negative. Ultimately, imaging investigations detected superior mesenteric artery syndrome as an etiologic background. In the additional investigations, it is found that she suffered from celiac disease. Conclusion. We concluded that the inflammatory nature of the celiac disease can affect the anatomy of the duodenum beyond its mucosal surface in the mesenteric fat tissue and results in SMAS.