Budd-Chiari syndrome (BCS) is a rare disease characterized by obstruction of the hepatic veins, regardless of mechanism and degree, in the absence of heart disease, pericardial disease or sinusoidal obstructive syndrome. In relation to etiology, BCS is classified in primary BCS, when it is caused by an intravascular thrombosis, and secondary BCS, when the obstruction is due to extrinsic compression or tumor invasion. The positive diagnosis is based on patient’s medical history, clinical examination, and paraclinical investigations. The clinical picture is polymorphous. The most common signs and symptoms include fever, abdominal pain and abdominal tenderness on palpation, hepatomegaly, ascites, edema of the lower limbs, gastrointestinal bleeding and hepatic encephalopathy. Laboratory tests can detect the degree of liver injury, with decreased serum albumin, altered coagulation, hepatic cytolysis and cholestasis, while imaging investigations may reveal the venous obstruction. Regarding treatment, all patients with BCS are recommended systemic anticoagulation to reduce the risk of thrombus extension and prevention of further thrombotic episodes. Depending on patient’s individual characteristics, angiography with or without stent, thrombolysis, trans-jugular intrahepatic portosystemic shunt and liver transplant are to be considered. The syndrome’s severity and prognosis are dictated by the underlying disease, ranging from a possible curability to quick death. Copyright