Chronic granulomatous disease (CGD) is a rare, genetically transmitted disorder characterized by recurrent, life-threatening infections with catalase-positive micro-organisms and excessive inflammatory reactions that lead to granuloma formation. Long-term prophylactic antimicrobial agents and aggressive surgical management are the mainstays of therapy. The authors provided anesthetics for 17 patients with CGD undergoing 55 surgical procedures. These patients presented to surgery with multiple organ system involvement and were at significant risk for complications in the perioperative period. Granulomatous lesions of the gastrointestinal (GI) tract may predispose such patients to regurgitation and aspiration.