A clinical case of a rare combination of complete transposition of internal organs (situs inversus totalis) with adenocarcinoma of the major duodenal papilla is presented. In addition to situs vicserum inversus, the patient has a special variant of vascular anatomy that is not included in the generally accepted classification of variants of arterial liver anatomy according to Michaels N.A. (1955), namely: separate separation of the left and right hepatic arteries from the ventral trunk. After individual preoperative planning, a patient with a complete reverse position of the abdominal organs and non-standard vascular anatomy was performed Whipple procedure. Non-invasive CT angiography is important in a complex preoperative examination, which allows timely identification and clarification of frequently occurring features of blood supply to the hepatopancreatoduodenal zone in patients with situs inversus totalis. Such surgical interventions should be performed in large multi-specialty medical centers that have extensive experience in performing pancreatoduodenal resections.