Badd-Chiari syndrome (BCS) is a rare disorder clinically characterised by pain abdomen, hepatomegaly and ascites. We observed 3 children with BCS. Histopathology of biopsy specimen of liver was diagnostic. One child had developed cirrhosis of liver because of long standing venous congestion. Inferior vena cavography (IVC-graphy) and percutaneous hepatography were helpful to localise the site and extent of obstruction. The cause of occulsion of hepatic vein (HV) and inferior vena cava (IVC) could not be identified. One child showed spontaneous improvement. Other two children had progressive disease. Early recognition of the syndrome can help the patient, in presence of operable causes.