In chronic granulomatous disease (CGD) patients, esophageal stricture is a rare complication and the treatment of choice is still controversial. There are few reports of successful therapy with antibiotics, corticosteroids, multiple balloon dilatations or their combination.We report a 3-three-year-old Iranian boy with recurrent esophageal obstruction due to CGD. The patient transiently responded to dilatation in one occasion and at another time to short term steroid therapy. We observed an excellent response when long term and high dose of corticosteroid was administered. It showed that a long term and high dose steroid therapy is more effective than a short term in a patient with CGD and esophageal stricture.