Intrahepatic cholestasis with situs inversus of the liver has not been reported in the literature. PURPOSE--A follow-up case for approximately 14 years (by one of the authors, MM). METHODS--Clinical aspects were studied and several laboratory exams as well as three ultrasonography and one computerized tomography exams were performed. Percutaneous and endoscopic cholangiographies were attempted. RESULTS--Four days after the patient was submitted to a surgical replacement of the aortic valve (with extracorporeal circulation), she developed jaundice, severe pruritus, dark urine, and pale stools. Serum levels of bilirubin, alkaline phosphatase, gama-glutamil transferase, and cholesterol were very high. Ultrasonography and computerized tomography exams did not reveal any evidence of extrahepatic obstruction. The tomography exam confirmed the existence of situs inversus in the stomach and liver. We were not successful in performing the cholangiography exams. Clinical and laboratory data as to cholestasis returned to normal after 4 months with no recurrence or complication during the follow-up period (14 years). CONCLUSION--The differential diagnosis between intra and extra-hepatic cholestasis in patients with situs inversus of the liver may be difficult. Therefore it will be necessary to collect clinical data and various complementary exams such as endoscopic retrograde cholangiopancreatography which is very difficult to perform in these patients.