Abstract Hepatoblastoma is the most common primary malignancy of the liver encountered in children. Primary tumors of the liver account for approximately 1% of malignancies in children. 50-60% of hepatic tumors in children are malignant, with >65% of these malignancies being hepatoblastomas. Hepatoblastoma occurs predominantly in children <3 yr of age. We present a 9 years old girl, who presented to our emergency ward with Jaundice and abdominal distension since 3 months. Examination showed pallor, icterus, hepatomegaly and ascites. Laboratory investigations revealed hyperbilirubinemia(TB:16.6, DB: 9) with thrombocytopenia (80000 cells/cumm). USG abdomen showed a mass lesion in right lobe of liver with irregular and nodular. CECT abdomen revealed a mass lesion of size 6.8x 5.5 x 5.9 cm in segment of right lobe with cirrhosis and metastatic nodules in the liver with moderate ascites and right pleural effusion. Spleen was moderately enlarged measuring 14 cm with infarcts. USG guided liver biopsy was done to confirm the diagnosis which showed histology pattern suggestive of Small Cell Undifferentiated Variant of Hepatoblastoma. Even a short period of history of jaundice and abdominal mass should rise the suspicion of hepatoblastoma, which help in early diagnosis and treatment.