Heterotaxy syndrome with polysplenia is a congenital abnormality with malposition of the visceral organs and multiple associated abnormalities. The majority of affected individuals die during childhood due to severe cardiovascular anomalies. However, affected individuals who reach adulthood are usually asymptomatic. Anomalies of the abdominal venous system, including interruption of the inferior vena cava with azygos or hemiazygos continuation, congenital absence of the portal vein with a portosystemic shunt, preduodenal portal vein, and abnormalities of renal veins are frequent findings. Heterotaxy syndrome usually has no clinical significance except in the setting of planned vascular intervention or surgery. We are reporting an extremely rare case of heterotaxy syndrome (polysplenia) that presented with massive variceal hemorrhage due to associated venous malformation and systemic to portal venous system collaterals.