Various benign and malignant conditions can cause biliary obstruction. We present a rare case of obstructive jaundice due to the compression of the common hepatic duct by the anteriorly overriding right hepatic artery. This case was also associated with the absence of the lateral segment of the left hepatic lobe. The patient was a 39 year-old housewife with a 4-day history of jaundice and occasional febrile sensation. An abdominal computed tomography showed absence of the lateral segment of the left hepatic lobe and a percutaneous transhepatic cholangiography showed a band-like filling defect of 2 mm width at the level of the upper common hepatic duct. The anteriorly overriding right hepatic artery compressing the common hepatic duct and the absence of the lateral segment of the left hepatic lobe were confirmed by operation.