We report a case of a 68-year-old female patient presenting with portal hypertension and variceal bleeding in the absence of any liver disease. After performing a computed tomography angiogram, the cause of her condition was identified to be a splenic arteriovenous fistula (SAVF). After confirming the findings with angiography, we opted to treat the condition with coil embolization as an alternative to a more invasive surgical treatment. Coil embolization of the SAVF was performed successfully resulting in the improvement of the patient's variceal congestion. Our case highlights the importance of identifying SAVF as a potentially curable cause of variceal bleeding in the absence of liver disease. Seeking this diagnosis is of utmost importance since it completely changes the endovascular approach and management of these patients with variceal bleeding. We describe a minimally invasive endovascular technique for treatment of these critically ill patients. © The Author(s) 2013.