We studied a cohort of 73 children exposed to cocaine in utero to characterize the spectrum of neurologic and ophthalmologic abnormalities; 36 children with no documented in utero drug exposure were selected as a control group. Most referrals of cocaine-exposed children to the child neurologist were made in the 1st year of life (81%). Reasons for referral were hypertonia (29%), seizures (22%), developmental delay (11%), tremulousness (11%), and hypotonia (4%). The most common abnormal finding in the cocaine-exposed children was hypertonia/hyperreflexia (63%), which was rarely seen in the control group. In addition, hypertonia/hyperreflexia was underdiagnosed by referring physicians. Similarly, hypotonia was seen on neurologic examination of cocaine-exposed children more frequently than documented by referral (16% versus 4%). Hypotonia was rarely seen in the control group. Twelve (43%) of 28 cocaine-exposed children seen by a pediatric ophthalmologist had structural ophthalmologic abnormalities. Neurologic and ophthalmologic findings suggesting structural lesions of the nervous system must be considered in cocaine-exposed children.