We sought to describe the clinical presentation and consequences of meningitis among 64 very-low-birth-weight (VLBW <1.5 kg) infants who had 67 culture-proven episodes of meningitis over an 18-year period, 1977 through 1995. Demographic and neonatal descriptors of meningitis and later outcomes were retrospectively examined and neurodevelopmental outcomes of 39 of 45 (87%) meningitis survivors were compared to those of nonmeningitis survivors followed up to 20 months corrected age. Causes of meningitis included coagulase-negative Staphylococcus in 43% of episodes, other gram-positive bacteria in 19%, gram-negative bacteria in 17%, and Candida species in 20% of episodes. Spinal fluid abnormalities were sparse, regardless of etiologic organism. Of 38 nonbloody spinal fluid taps (<1,000 erythrocytes/mm3), 6 had >30 leukocytes/mm3, 5 protein >150 mg/dL%, and 6 glucose <30 mg/dL (1.67 mmol/L). Only 10 infants (26%) had 1 or more of these spinal fluid abnormalities. Meningitis survivors had a higher rate of major neurologic abnormality (41% vs 11%, p<0.001) and subnormal (<70) Mental Development Index (38% vs 14%, p<0.001) than nonmeningitis survivors. Impairment rates did not differ by etiologic organism. The effect of meningitis on neurologic outcome persisted even after controlling for birth weight, intraventricular hemorrhage, chronic lung disease, and social risk factors (odds ratio 2.27 [95% CI 1.02, 5.05]). We conclude that despite a sparsity of abnormal spinal fluid findings, culture-proven neonatal meningitis among VLBW infants has a detrimental effect on neurologic outcome, which persists even after controlling for other risk factors.