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Background: Vascular disorders of the liver are relatively rare in clinical practice. Budd Chiari syndrome (BCS) is a potentially life-threatening condition. It describes a group of disorders characterized by hepatic venous outflow obstruction at any level from the small hepatic veins up to the junction of the inferior vena cava and the right atrium. The prevalence of Budd Chiari Syndrome is greatly influenced by geographical differences. There is a dearth of data in Nigeria regarding this condition. Case report: We report the case of a thirty seven year-old woman presenting with an eight year history of recurrent abdominal swelling, jaundice and progressive weight loss. The patient was promptly screened for viral hepatitis B and C, which were both negative. The laboratory work-up revealed mild hepatic dysfunction and an abdominal ultrasound scan showed mild hepatomegaly and ascites. Abdominal Computed Tomography (CT) scan findings were consistent with Budd Chiari Syndrome, based on the findings of an extensive thrombus, involving the common iliac and hepatic veins, in addition, the inferior vena cava (IVC) and right atrium. Conclusion: This case report highlights the importance of the knowledge of Budd Chiari Syndrome and the diagnostic approach. This condition should be considered whenever investigating the aetiology of chronic or acute liver disease in Gastroenterology practice in Nigeria. [ABSTRACT FROM AUTHOR]