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Chronic septic granulomatosis is a rare genetic immune deficiency resulting from dysfunction of the nicotinamide adenine dinucleotide phosphate oxidase (NADPH oxidase) complex in phagocytic cells.Clinical manifestations fall into two broad categories: infectious and inflammatory. Infections are the most common manifestations of the disease at any age, while inflammatory conditions are usually confined to adults.This is a serious disease. Management is mainly based on hygienic-dietary measures and chemo-prophylaxis to prevent fungal and bacterial infections.Life expectancy has improved markedly, but the prognosis may be jeopardized by infectious and inflammatory complications.