We report the case of a 39-year-old woman with portal hypertension caused by a splenic arteriovenous fistula that was diagnosed by Doppler ultrasound and splenic arteriography. She presented with esophageal variceal hemorrhage and was initially treated with sclerotherapy. Ascites and secretory diarrhea then developed. At laparotomy portal pressure was 60 cm H2O but fell to 26 cm H2O after the fistula was resected with a splenectomy. All symptoms disappeared shortly thereafter, and the patient has remained well for the past two years.