Transmesenteric hernia is a rare cause of bowel obstruction and strangulation. It can be due to iatrogenic injury, trauma, inflammatory and less likely congenital. We present a case of true congenital transmesenteric hernia in a 26-year-old male with no previous surgical history or trauma. The patient presented acutely with no prior symptoms. Investigations provided limited information towards the diagnosis of the hernia, but suggestive of mid-gut volvulus. Emergency laparotomy was performed to find >75% of small bowel herniating through a proximal jejunal mesenteric defect leading to incarceration and early strangulation. No resection was required and patient recovered well. This case presents yet another rare pathology and highlights the importance of keeping a low threshold for operative intervention in uncertain cases.