The case of a 73-year-old man with localized left-lower-limb hypertrophic osteoarthropathy in association with aortic left-iliac-artery bypass and enteroprosthetic fistula was studied. The patient presented first with massive gastrointestinal bleeding and with pain and swelling of his left leg. He was hospitalized on numerous occasions for recurrent episodes of bacteremia involving enteric flora; hypertrophic osteoarthropathy and aortoduodenal fistula were documented before surgery. Symptoms and signs of hypertrophic osteoarthropathy were markedly alleviated after removal of the infected prosthesis. A review of the literature revealed seven additional patients with a similar presentation. The diagnosis of infected arterial graft with enteroprosthetic fistula may be extremely difficult, and a delay in this diagnosis may be lethal. Awareness of this uncommon association may lead to prompt diagnosis and early surgical therapy.