Congenital transmesenteric defect is a rare but serious condition which presents a diagnostic challenge as symptoms and investigations are nonspecific. Here, we present a case with fetal ascites and dilated bowel loops who underwent intervention within 6 h of birth. Laparotomy showed volvulus and gangrene of 45 cm of the distal ileum, which was herniating through mesenteric defect. Timely detection and intervention can help in preventing significant morbidity and mortality. Given the downside to delay of surgical intervention and lack of specific tests, open exploration continues to be the only way to establish a definitive diagnosis of a transmesenteric hernia.