Background: Wilkie syndrome (WS) is a pathological manifestation caused by an abnormal disorder of the superior mesenteric artery (SMA). It is a branch of the abdominal part of the aorta. As a result, the distal part of the duodenum is compressed between the abdominal aorta, spine, and SMA, which creates a hindrance for the passage of chyme in the duodenum, creating a mechanical obstruction, and thus, gives rise to the WS. It is commonly due to the loss of fatty tissue. Case Presentation: We hereby discuss the case report of a 28-year-old patient diagnosed with the same at the initial stage of WS. The patient had been suffering from the symptoms of WS since 2018 but wasn’t able to identify the disease when he came to our department. Based on the results of abdominal sonography, Computed tomography (CT)-scan with contrast, and angiography, we confirmed the diagnosis of WS. The patient was treated with the duodenojejunostomy (laparoscopic Strong’s operation), and after the surgery, the patient was treated symptomatically. Conclusion: WS is a rare and fatal manifestation that if delayed can cause severe life-threatening complications and even death, but diagnosis in the initial stages, especially with contrast CT/magnetic resonance imaging angiography, and timely management can save the life and prevent fatal complications. WS can be a fatal manifestation if not diagnosed on time, but early and accurate diagnosis can unleash the diseased state of WS, and treatment on time can avoid severe and deadly complications.

Keywords: Wilkie’s syndrome, laparoscopic Strong’s operation, aorto-mesenteric angle and distance, nutcracker syndrome, case report.