Budd-Chiari syndrome (BCS) (artery-occlusive hepatic disease) is a rare disease characterized by the obstruction of the blood flow at the level of the suprahepatic veins till their flow in the inferior vena cava (IVC) or at the level of IVC on the segment between the suprahepatic veins and the right atrium. The most frequent symptoms are abdominal pains, hepatomegaly and ascites. The imagistic investigations have an essential role in the early establishment of the diagnostic, evaluating the extension of disease and the management of BCS. Treatment depends on the presence or absence of symptoms and how acute the disease is.We present the case of a 43-years old woman, who had had for two month dyspeptic symptoms, increase of volume of the abdomen and oedema in the lower limbs. The biological investigations indicated hepatic dysfunction and thrombocytosis. The abdominal ultrasound showed modifications of chronic hepatopathy with signs of portal hypertension. The abdominal computer-tomography emphasized hepatomegaly with multiple nodules of regeneration, signs of portal hypertension (splenomegaly, moderate ascites), caudate lobe hypertrophy and thrombosis IVC. The patient was diagnosed with BCS and essential thrombocythemia. She started the medical treatment and was listed for liver transplantation. Budd-Chiari syndrome has to be taken into account every time we investigate the etiology of an acute or chronic hepatopathies, because the early diagnose can improve the patient�s prognostic.