Hepatoblastoma (HB) is a rare malignant tumor of the liver; but is the most common malignancy of liver in infancy and childhood that usually occurs in the first three years of life. These tumors are usually present by abdominal mass, abdominal pain, anemia, thrombocytosis, and high serum levels of AFP; and may cause decreased appetite and weight loss.
Complications such as ascites, GIB, icterus, splenomegaly and vascular thrombosis may occur, while hepatic vein or inferior vena cava thrombosis is quite rare especially with a spread to right atrium and pulmonary artery branches. Multidisciplinary therapy by a pediatric oncologist, a pathologist, an experienced radiologist, a pediatric cardiologist and a pediatric surgeon is very important to diagnose and manage the condition. We report on the case of an infant with a huge abdominal mass and much extended tumor thrombosis till the pulmonary artery branches that shrunk appropriately after the first course of chemotherapy.