Meningitis due to Candida albicans was successfully treated in a 1.1 kg premature infant using combined antifungal therapy of amphotericin B for three weeks and 5-fluorocytosine for four months. Hydrocephalus and profound psychomotor retardation were present one year later. Psychomotor retardation, aqueductal stenosis and hydrocephalus were found to be common in a review of 16 previously reported cases of central nervous system (CNS) candidiasis in newborn infants. The diagnosis and institution of therapy were frequently delayed, and the mortality rate was 29% in the 17 patients reviewed here. The subacute course, lack of clinical findings, variable cerebrospinal fluid (CSF) findings, negative CSF cultures due to low concentrations of organisms, slow in vitro growth of C. albicans and misinterpretation of positive cultures as contaminants are factors frequently leading to delayed diagnoses. Using combination therapy, it should be possible to use lower doses and shorter courses of amphotericin B therapy for C. albicans meningitis in the newborn infant.