Anatomical abnormalities of the liver are extremely rare. Although the majority of cases with an accessory liver are not detected, it can give rise to various clinical symptoms like recurrent abdominal pain and impaired liver function. Here we present the first case of orthotopic liver transplantation in a patient with hepatic ischemia caused by complete vascular occlusion due to a twisted accessory liver lobe. Although rare, an accessory liver lobe may cause serious and life-threatening problems and should therefore be kept in mind in patients presenting with acute abdominal pain.