Hyperimmunoglobulin E syndrome is a relatively rare primary immunodeficiency syndrome characterized by recurrent infection, abscess formation and marked elevation of serum IgE level. The common infectious organism is Staphylococcus aureus and recurrent infection indicates some defects in the immunologic system. Although the infection can affect various organs, gastrointestinal tract involvement is rare and only one case of colon perforation has been previously reported. Herein we report another one case of colon perforation which ocurred in an 8-year-old girl with hyperimmunoglobulin E syndrome. The patient was admitted to the hospital due to an abscess on right neck. The diagnosis of hyperimmunoglobulin E syndrome was made because she had eczematoid dermatitis on the face, pneumatocele on left upper lung field and markedly elevated serum Ig E level(> 15,000 IV/ml) with a past histories of frequent scalp abscesses and otitis media. Abdominal pain developed on the 13th day of admission and abdominal plain X-ray revealed free air. An exploratory laparatomy was performed and two free perforations of the transverse colon were noted. Segmental resection and double barrel colostomy were performed. Colostomy closure was done 4 month later and she had no gastrointestinal problem during a follow up period of 15 months.