A Taiwanese boy was diagnosed as having hyperimmunoglobulin E syndrome at the age of 4 years. At age 18 he was admitted to the hospital because of pneumonia in the left lower lobe. Abdominal pain developed 9 days later, and his condition progressed to peritonitis. Colon perforation, 10 cm distal to the ileocecal valve, was found. Double-barrel ileostomy was performed, and reanastomosis was done 1 1/2 months later. Afterward, he was fine, and he had no significant gastrointestinal problems after being discharged. To the author's knowledge, this is the first reported case of hyperimmunoglobulin E syndrome complicated by colon perforation.