BACKGROUND: Chronic granulomatous disease (CGD) is an uncommon congenital 
phagocyte disorder characterized by recurrent life-threatening infections. CGD 
generally present with recurrent suppurative infections, however, intracranial 
fungal abscess complicating CGD may cause a diagnostic problem to anyone 
unfamiliar with its clinical and radiological features.
HISTORY: We report the case of a 16-year-old boy who was consulted with a 
differential diagnosis of an intracranial tumor. The clues of his medical 
history and physical examination made us consider the diagnosis of CGD. 
Cytometric dihydrorhodamine assay and genotyping confirmed an autosomal 
recessive CGD. He was successfully treated without any complication or sequel 
for 18 months follow-up period with surgery and interferon-gamma, in addition 
with, liposomal amphotericin B and voriconazole that were found to be sensitive 
to the Aspergillus fumigates, which had been grown from the culture of the 
abscess cavity.
DISCUSSION: We discuss the pathogenesis, radiological techniques, and management 
of cerebral Aspergillus abscess in a patient with CGD.
CONCLUSION: Presentation of CGD with a cerebral Aspergillus abscess, mimicking a 
brain tumor is extremely rare in children; clinicians and neurosurgeons must be 
aware. The best management modality for cerebral Aspergillus abscess is to be 
vigilant about the disease, whereas adjuvant surgical and medical therapy with a 
close follow-up must be warranted for all cases.