Chronic granulomatous disease (CGD) is a rare inherited disorder characterised by inability of phagocytes to kill catalase-positive organisms including certain fungi. Aspergillus species are the most frequent fungal pathogens. This study is a systematic review of the reported cases of osteomyelitis due to Aspergillus species in CGD patients. Retrospective analysis of 46 osteomyelitis cases caused by Aspergillus species in 43 CGD patients (three females) published in the English literature (PubMed) was performed. Twenty-three cases were due to Aspergillus fumigatus (50%), 20 to Aspergillus nidulans (43.5%), one to Aspergillus flavus and two to unspecified Aspergillus species. The median age was 8 years (range 1.5-21). Osteomyelitis due to A. nidulans was associated with pulmonary infection and involved 'small bones' more frequently than A. fumigatus osteomyelitis (P = 0.001). Amphotericin B was used in 91.3% and surgical debridement in 67.4% of all cases. The overall mortality of osteomyelitis due to Aspergillus species in CGD patients was 37%; 55% for A. nidulans compared to 13% for A. fumigatus (P = 0.008). Aspergillus fumigatus causes osteomyelitis in CGD patients almost as frequently as A. nidulans and much more frequently than A. flavus. Osteomyelitis due to A. nidulans is associated with higher mortality than A. fumigatus.