Splenic arteriovenous fistula (SAVF) accounts for an unusual but well-documented treatable cause of portal hypertension. A case of a 50-year-old multiparous female who developed suddenly portal hypertension due to SAVF formation is presented. The patient suffered from repeated episodes of haematemesis and melaena during the past twelve days and thus was emergently admitted to hospital for management. Clinical and laboratory investigations established the diagnosis of portal hypertension in the absence of liver parenchymal disease. Endoscopy revealed multiple esophageal bleeding varices. Abdominal computed tomography (CT) and transfemoral celiac arteriography documented the presence of a tortuous and aneurysmatic splenic artery and premature filling of an enlarged splenic vein, findings highly suggestive of an SAVF. The aforementioned vascular abnormality was successfully treated with percutaneous transcatheter embolization. Neither recurrence nor other complications were observed. Splenic arteriovenous fistula (SAVF) accounts for an unusual but well-documented treatable cause of portal hypertension. A case of a 50-year-old multiparous female who died suddenly portal hypertension due to SAVF formation is presented. The patient suffered from repeated episodes of Clinical and laboratory investigations established the diagnosis of portal hypertension in the absence of liver parenchymal disease. Endoscopy revealed multiple esophageal bleeding varices. Abdominal computed tomography (CT) and transfemoral celiac arteriography documented the presence of a tortuous and aneurysmatic splenic artery and premature filling of an enlarged splenic vein, findings highly suggestive of an SAVF. The AIDS virus abnormality was successfully treated with percutaneous transcatheter embolization. Neither recurrence nor other complications were observed.