The range and extent of neurologic and neurobehavioral complications of human immunodeficiency virus (HIV-1) infection in children are under-described. Seventy-eight children with HIV-1 infection (32 females) were assessed for neurologic complications. Forty-six children had abnormal neurology examinations. Thirty-three children had global pyramidal tract signs, 5 had a hemiparesis, 4 had peripheral neuropathy, 18 had visual impairment, and 5 had hearing impairment. Thirty-nine of 63 children over 1 year of age had neurobehavioral problems. Of 24 children with HIV encephalopathy, 74% had severe immunosuppression and 45% were not receiving antiretroviral therapy. Twelve children had prior opportunistic central nervous system infections, and 9 had epilepsy. Diverse neurologic and neurobehavioral deficits are common in children with HIV-1 infection. Children with severe immunosuppression, who were not receiving antiretroviral therapy, were growth impaired and less than 1 year of age, were at greatest risk for developing neurologic complications.