CGD is an immunodeficiency caused by mutations in genes encoding subunits of the NADPH oxidase complex. Normally, assembly of the NADPH oxidase complex in phagosomes of phagocytic cells leads to a “respiratory burst” essential for the clearance of microorganisms. CGD patients lack this mechanism, which results in life-threatening bacterial and fungal infections and granuloma formations. The leading cause of death are pneumonia and pulmonary abscess, septicemia and brain abscess. In neurogical manifestations various pathogens have been involved including Aspergillus spp., S. prolificans, A. infectoria, Salmonella and Staphylococcus spp. There are only some several reports on fungal brain and spinal cord infection due to Candida spp. To decrease mortality and morbidity from fungal infections the prophylactic use of itraconazole or voriconazole is widely recommended. A relatively new azole, posaconazole is active in pulmonary and cerebral fungal manifestations, indeed may be effective against fungi with inherent resistance to AmpB or voriconazole. In the past twenty years we have managed seven children with CGD. We present a two – year history of an X-linked CGD patient with brain abscess. In spite of our effort we were unable to identify any causative pathogen. The brain abscess did not respond to conventional antibacterial and antifungal treatment for a long time. Based on the findings and literature data we presumed the causative agent might be some kind of moulds. We suppose the use of echinocandin and posaconazole as salvage (”prophylactic”) therapy. It has resulted significant regression of the brain abscess.