Abstract Chronic granulomatous disease (CGD) is a rare inherited disorder of host defense, in which the immune system’s phagocytes are incapable of producing peroxide radicals, which are usually employed by phagocytes to kill microorganisms. These phagocytes display impaired antimicrobial activity, and the patients suffer from recurrent and frequently fatal bacterial and fungal infections. We present an unusual case of an adolescent with fever, cough, and dyspnea, as well as a medical history of recurrent pneumonia and multiple hospitalizations. Diagnostic findings include abnormal values of nitroblue tetrazolium and dihydroergotamine suggestive of CGD. This case highlights the importance of the multidisciplinary approach in prompt diagnosis and long-term care of CGD patients.