Budd-Chiari syndrome is a rare disease defined by the obstruction of hepatic venous outflow anywhere from the small hepatic veins to the junction of the inferior vena cava and the right atrium. This syndrome is uncommon in children. The article presents a case report of chronic refractory Budd-Chiari syndrome with a leading ascites in 16 years adolescent, which required surgical intervention--transjugular intrahepatic porta systemic shunt. As this syndrome is uncommon in pediatric practice, complex differential diagnostic search and delays in the diagnosis are frequent. This case report emphasizes the importance of a high index of suspicion in the diagnosis of pediatric Budd-Chiari syndrome and highlights the importance of early referral to a specialized pediatric unit for the further treatment.