Internal hernias (IHs) are defined as the protrusion of the intestine through an aperture in the peritoneal ligament, mesentery, or the omentum, without traversing the fascial planes and leading to encapsulation of the protruded intestine within another compartment of an otherwise intact abdominal cavity. Internal hernias can be acquired or congenital. Congenital internal hernias resulting in small bowel obstruction are extremely rare, even more so in adults, occurring usually due to embryological or developmental defects, often undiagnosed, and requiring a high index of clinical suspicion. We report a rare case of a 22-year-old young adult with transmesenteric hernia of distal ileum through a congenital distal ileal mesenteric defect resulting in closed-loop small bowel obstruction and distal ileal gangrene, managed with resection and end-ileostomy, followed by stoma reversal one month later. It requires a high index of suspicion and remains a surgical emergency due to its predilection for intestinal gangrene and small bowel obstruction, with a high mortality rate due to delayed presentation and diagnosis.