An intrahepatic portosystemic venous shunt is a rare condition that was initially reported by Doehner et al. in 1956. In addition, the occurrence of midgut malrotation is rare in adulthood. The association of a portosystemic shunt along with a midgut malrotation and a renal rotational anomaly has not yet been described. We report a case of an intrahepatic portosystemic shunt, midgut malrotation, and renal rotation anomaly.