In the United States, most cases of portal hypertension result from intrinsic liver disease. However, when no liver disease is obvious, rarer etiologies must be carefully evaluated. Here we discuss the case of a splenic arteriovenous fistula (AVF) that led to severe portal hypertensive bleeding and gastrointestinal ischemia. A 34 y/o black female with no significant past medical history presented to our institution with 10 days of watery diarrhea and diffuse post-prandial abdominal pain. 5 days prior, she went to an outside hospital with these same complaints, where she was given a diagnosis of gastroenteritis and sent home with PPI. In our ER she underwent CT scan, which showed diffuse bowel wall thickening and significant portal hypertension (esophageal, gastric, and rectal varices and enlarged splenic and portal veins). She had no history of or significant risk factors for liver disease. While awaiting admission, she had hematemesis and hematochezia with a change in her hemodynamic status. Emergent endoscopy revealed Grade III esophageal varices, ischemic-appearing stomach and duodenum, and a large fundal pool of blood with pulsatile arterial spurting. After control of bleeding with epinephrine injection and evacuation of the fundal pool, large gastric varices were noted, but the bleeding had subsided. Given these findings, balloon tamponade of the gastric varices was achieved with a Minnesota tube. The CT and subsequent liver duplex failed to reveal evidence of portal venous ouflow obstruction. Urgent angiography was peformed to further evaluate the “idiopathic” portal hypertension. This revealed a large splenic AVF, treated with the placement of multiple coils in the mid-splenic artery. After the procedure she had no further bleeding, pain, or diarrhea and was tolerating a full diet. Repeat CT showed improvement in the bowel wall thickening. She was discharged home with surgical follow-up for consideration of splenectomy. Splenic AVF is a rare cause of forward portal hypertension in association with mesenteric ischemia. Patients can present with complications of portal hypertension, as seen in our patient. The diagnosis may be difficult to make without arteriogram. On CT scan early filling of the portal vein on the arterial phase can give a clue to this diagnosis and was seen in retrospect in our patient. The diagnosis of splenic AVF should be considered in the differential diagnosis of portal hypertension and mesenteric ischemia, especially in the absence of liver disease.