Acquired hepatocerebral degeneration (AHD) is an extrapyramidal syndrome that develops secondary to hepatic dysfunction. It may accompany advanced liver disease and is related to the presence of portosystemic shunts. We describe a 44-year-old woman who presented with bleeding from gastric fundus varices secondary to splenic vein thrombosis and concomitant development of rapidly progressive extrapyramidal symptoms. No overt liver dysfunction was identified. Brain imaging demonstrated damage to the basal ganglia. The splenic artery was embolized with disappearance of the varices. Medical treatment consisted of amantadine and low-dose clonazepam for 6 months. The patient's neurologic symptoms gradually improved with few sequelae, although imaging demonstrated residual basal ganglia damage. This case highlights the need to identify reversible causes of AHD and treat them rapidly in order to halt progressive damage. The general prognosis of patients with AHD remains poor, and flow interruption in portosystemic shunts must always be taken into account. Rapid diagnosis and treatment could ameliorate the poor prognosis.