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BCS patients often have a combination of prothrombotic risk factors. Aetiology and presentation differ between Western and certain Asian countries. Myeloproliferative neoplasms are present in 35%\u201050% of European patients and are usually associated with the JAK2\u2010V617F mutation. Clinical presentation is diverse and BCS should be excluded in any patient with acute or chronic liver disease. Non\u2010invasive imaging (Doppler ultrasound, computed tomography, or magnetic resonance imaging) usually provides the diagnosis. Liver biopsy should be obtained if small vessel BCS is suspected. Stepwise management strategy includes anticoagulation, treatment of identified prothrombotic risk factors, percutaneous revascularisation and transjugular intrahepatic portosystemic stent shunt to re\u2010establish hepatic venous drainage, and liver transplantation in unresponsive patients. This strategy provides a 5\u2010year survival rate of nearly 90%. Long\u2010term outcome is influenced by any underlying haematological condition and development of hepatocellular carcinoma.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>With the advent of newer treatment strategies and improved understanding of BCS, outcomes in this rare disease have improved over the last three decades. 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