Chronic granulomatous disease (CGD) is a disease in which a granulomatous p
rocess 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 g
ranulomata. The patient responded well to a CSF shunt and antifungal therap
y.