Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) is associated with arteriovenous fistulas throughout the body that can cause hemodynamic abnormalities. Owing to their size and extent, surgical repair is often not feasible. A patient referred for presumed valvular heart disease is described. On the basis of oximetry data at cardiac catheterization, a large intrahepatic arteriovenous fistula was discovered by aortography. This finding and a history of recurrent epistaxis were consistent with Osler-Weber-Rendu disease. Owing to the size of the fistula, embolization of the right hepatic artery with Gianturco coils was chosen as treatment, with resultant symptomatic improvement and decreased arteriovenous shunting.