Introduction and Importance: Internal hernia is responsible for 0.6–5.8% of all small intestinal obstructions. Only 8% of internal hernias are of the congenital trans mesenteric variant. Urgent surgical intervention should be considered in individuals who exhibit intestinal obstruction before the development of irreversible bowel ischemia and necrosis. Case Presentation: The authors report a 38-year-old male patient who presented to the emergency department with abdominal pain, distension, and vomiting for the last three days. After an explorative laparotomy, it was confirmed that there was a trans mesenteric hernia defect with strangulated distal ileal loops. End-to-end ileo-ileal anastomosis was done. Clinical Discussion: Early recognition and subsequent surgical treatment permit proper management and prevent complications. There should be a differential diagnosis. In this case, there is no prior history of abdominal surgery, and the patient presents with recurrent abdominal pain and intestinal obstruction. Conclusion: Early diagnosis and emergency laparotomy can save the intestine before gangrene, lowering morbidity and mortality, correcting the mesenteric defect to prevent recurrences, and enhancing clinical outcomes because many studies have shown that some cases are missed before radiological investigation. Laparotomy is still the method of choice for acute cases of incarceration with bowel obstruction, strangulation, and ischemia. The entire mesentery needs to be evaluated, and all mesenteric defects need to be sutured to prevent recurrence.