Hepatodiaphragmatic interposition of the intestine, known as Chilaiditi’s syndrome, is a rare and often asymptomatic anomaly, typically found as an incidental radiographic sign. We report a case of Chilaiditi’s syndrome associated with transverse colon volvulus, predisposed by segmental agenesis of the right lobe of the liver. A 45-year-old man presented with a 2-day history of abdominal pain, nausea, vomiting, and constipation. Plain chest X-ray and abdominal computed tomography showed colonic interposition and segmental agenesis of the right lobe of the liver. Laparotomy revealed a clockwise volvulus of the transverse colon associated with interposition and incarceration of the colon through the space of the agenetic segment of the liver. The transverse colon, which was adherent to the agenetic space in the liver and diaphragm, was dissected away and repositioned, and the volvulus was reduced. To our knowledge, this is only the sixth reported case of a colonic volvulus associated with Chilaiditi’s syndrome and the first case associated with segmental agenesis of the right lobe of the liver.