Situs inversus totalis (SIT) is a rare condition, in which there is transposition of the thoraco-abdominal organs. This situation leads to diagnostic and therapeutic difficulties in patients with acute surgical abdomen. The objective of this report is to present the case of a patient who presented with colonic pain in the epigastrium and left hypochondrium, in which the diagnosis of SIT, cholelithiasis and choledocholithiasis was reached after the respective imaging studies. Once the diagnosis was made, treatment was performed through retrograde endoscopic cholangiopancreatography (ERCP) and subsequent surgical exploration of bile ducts. Both procedures were failed due to technical difficulties generated by the patient's condition. In conclusion, in spite of the report of successful cases in the literature of ERCP and surgical treatment of the biliary tract in SIT, there may be situations that do not allow a successful approach, so meticulous surgical planning and the use of support methods are necessary In the management of these patients.