A 3-year-old girl with the diagnosis of chronic granulomatous disease (CGD) was hospitalized for bron-chopneumonia and congestive heart failure. Her medical history included methylprednisolone medication for autoimmune gastric outlet obstruction. Computed tomography revealed pneumonic infiltrations and pericardial thickening. A pulsed-wave Doppler recording revealed E/A > 1. During a pericardiectomy, multiple islands of thick, firm-walled, fibrinous exudate-containing, small abscess formations were observed. Histopathological evaluation of pericardial tissue revealed granulomatous inflammation. Aspergillus fumigatus was cultured from the abscess. In conclusion, development of constrictive aspergillus pericarditis should be considered in patients with CGD because immediate initiation of antifungal management with aggressive surgical treatment is life-saving.