Budd-Chiari Syndrome (BCS) is a rare pathological condition characterized by an obstruction of the hepatic venous outflow. It can be classified as primary, due to an intra-luminal venous lesion, or secondary, resulting from extrinsic compression or invasion of the venous system. This study aims to report a case of Budd-Chiari Syndrome, emphasizing the importance of diagnosis and treatment in preventing progression to chronic liver disease. We report the case of a 47-year-old patient who presented to the emergency department with severe pain in the right hypochondriac region and abdominal distension. A total abdominal CT scan was performed and revealed thrombosis of the suprahepatic veins, confirming the diagnosis of BCS. The patient developed portal hypertension and signs of chronic liver disease. Treatment was administered with Spironolactone and Marevan. After six months of Marevan use, the patient experienced worsening of the esophageal varices and hematemesis, requiring esophageal variceal band ligation. Liver transplantation was not recommended as the patient's MELD score was 14. Therefore, an accurate diagnosis of BCS is crucial for appropriate therapeutic intervention in order to prevent the progression to chronic liver disease, with permanent damage to liver functionality and the patient’s quality of life.