Portal hypertension is a syndrome characterized by an elevation in pressure within the portal venous system, with severe consequences. Hepatic cirrhosis is its most common cause, but there are less frequent causes, including the recently described hepatic vascular disease, portosinusoidal vascular disease (PSVD), which affects portal venules and hepatic sinusoids. We present the case of a 68-year-old man with a history of portal hypertension and recurrent variceal bleeding. Despite medical treatment and endoscopic procedures, the hemorrhages persisted. A transjugular intrahepatic portosystemic shunt (TIPS) was performed to manage the condition. Hepatic elastography revealed unusually low liver stiffness for cirrhosis, prompting a liver biopsy. Histological findings supported the diagnosis of PSVD. Regarding treatment, there are no specific therapies for PSVD, and the focus is on managing complications associated with portal hypertension. PSVD is a rare entity that can be an underlying cause of non-cirrhotic portal hypertension. This case underscores the importance of awareness for early diagnosis and appropriate management. Ongoing research is crucial to gain a better understanding of this rare condition and enhance the quality of life for affected patients.