Budd-Chiari Syndrome is characterized by obstruction to the outflow of the liver due to the occlusion of the hepatic vein and sometimes the inferior vena cava. This syndrome comprises hepatomegaly, abdominal pain, ascites and hepatic histology showing zone 3 sinusoidal distention and pilling. It may, in the long run, progress to cirrhosis while, on the other hand, cirrhosis due to other causes, especially viral causes may progress to carcinoma and lead to Budd-Chiari Syndrome by compressing the hepatic vein. Here we present a 7-year old boy with a previously asymptomatic hepatitis B virus related hepatocellular carcinoma developing acute Budd-Chiari syndrome. He also had portal vein thrombosis along with acute hepatic decompensation which is uncommon worldwide. DOI: http://dx.doi.org/10.3329/seajph.v3i2.20043 South East Asian Journal of Public Health Vol.3(2) 2013: 66-69