A19-year-old woman with a medical history of bronchiectasis underwent computed tomography (CT) of the thorax. CT findings of tuberculosis, bronchiectasis and mediastinal lymph nodes were present on CT scan at the time of admission. Abnormalities such as hyparterial bronchi and absence of middle lobe fissure were seen on the examination suggesting heterotaxy syndrome. Hence, further abdominal CT examination was performed to show other accompanying anomalies. The abdominal CT revealed a normally positioned left-dominant liver. The pancreas was normally located, i.e. in the midline, with agenesis of the pancreatic tail and lateral part of the body. There were multiple spleens without positional anomaly on CT scan. The abdominal IVC was in the normal position, right to the midline, and continous with intrahepatic part draining into the right heart. The superior mesenteric vein (SMV) joined to the splenic vein after passing between the superir mesenteric artery (SMA) and aorta, and then it formed the main portal vein lateral to the SMA. The main portal vein was transversing the left and hypoplasic right liver lobes horizontally from midline to the lateral contour of the liver and entered the left liver lobe after making a u-turn (Figure 1A-1C). The main portal vein was bifurcating in the left-dominant liver.