Retropharyngeal neuroblastoma is rare. We report a 3-month-old infant with retropharyngeal neuroblastoma presenting with airway compression, which had an unresectable localized tumor without N-myc amplification. He was promptly treated with chemotherapy, resulting in a dramatic resolution. Subsequently, he received no surgical intervention and is well without evidence of recurrence 10 months after completion of chemotherapy. A review of the literature reveals that retropharyngeal neuroblastoma in infants has a good prognosis, but with some risk of surgical complication. Thus, it might be better to treat unresectable, localized disease accompanied by life-threatening symptoms with chemotherapy alone.