CNS granulomatosis in a child with chronic granulomatous disease

Chronic granulomatous disease (CGD) is a disease in which a granulomatous process involves various organ systems and in which recurrent infections are seen. The basic defect is the absence of the granulocyte respiratory burst. CNS involvement is rare. We present a report of a child with CGD and CNS involvement, presenting with hydrocephalus. Candida was identified in the granulomata. The patient responded well to a CSF shunt and antifungal therapy.