Chronic granulomatous disease (CGD) is a rare disorder with the unifying characteristics of severe predisposition to bacterial and fungal infections, impaired ability of phagocytic leukocytes to produce microbicidal oxygen metabolites and failure of these cells to kill certain microorganisms. Aspergillus spp. are the most common fungal pathogens in these patients. Cranial aspergillosis is a rare presentation with a high mortality rate. Immunoglobulin levels of CGD patients are usually normal or elevated. We herein describe a five-year-old boy with CGD presenting with disseminated aspergillosis and hypogammaglobulinemia (low IgA and IgG).