A patient was diagnosed with gastric outlet obstruction (GOO) 17 months after the neonatal diagnosis of chronic granulomatous disease (CGD). Gastric outlet obstruction was the first clinical manifestation of CGD in this patient. Twenty-three percent of the 17 patients with GOO complicating CGD described in the literature were found to present with GOO before any other clinical manifestations of CGD. The diagnosis of GOO can be established by ultrasound and, if necessary, upper gastrointestinal roentgenogram or gastroscopy. A nonsurgical approach to management is suggested. The diagnosis of CGD should be considered in an infant or child who has GOO.