Hereditary hemorrhagic telangiectasia is an inherited disorder in which patients may have multiple telangiectases and arteriovenous fistulas in the skin and internal organs. Patients can suffer from a variety of serious clinical complications, including abscess formation. We report two patients in whom neurologic symptoms developed from embolic abscesses, one for whom this complication was fatal. The reported incidence and microbiologic features of this complication are similar to that of endocarditis in patients with valvular heart disease. We believe that patients with hereditary hemorrhagic telangiectasia should receive similar antibacterial prophylaxis for procedures placing them at risk for bacteremia.