Colon perforation from hyperimmunoglobulin E syndrome is very rare, and only one case has been reported in the English-language literature. Herein, the authors report another case of colon perforation experienced in hyperimmunoglobulin E syndrome. The patient was an 8-year-old girl with frequent infection, eczematoid dermatitis, and an increased serum level of immunoglobulin E. During admission, panperitonitis developed caused by colon perforation. Treatment was resection of the perforated segment of the colon and a double-barrel colostomy. The patient has been doing well 18 months after treatment.