Background: In the pre-antiretroviral era, the frequency of neurologic complications was associated with low baseline CD4+ T-cell counts. Introduction of antiretroviral therapy (ART) has largely decreased the incidence of opportunistic infections and CNS neoplasia in the recent two decades; however, virus replication persists in the cerebrospinal fluid (CSF) and neuronal tissues due to variable drug penetration as well as development of drug resistance. Although many previous studies have addressed the presence of neurologic manifestations in the course of HIV infection; an update on the type of neurologic involvement, presenting signs and symptoms, radiologic findings, and response to treatment is essential. Methods: In a case series, we recruited 42 patients presenting with neurologic symptoms/signs and concomitant HIV infection in 12 months during 2015 and 2016 at a tertiary academic hospital. Information regarding the course of diagnosis, laboratory findings, radiologic findings, and final diagnosis were documented and analyzed in relation to the survival status of each patient during up to one month of hospitalization. Results: The mean age of the patients was 39.7; 25 were men, 19 were newly diagnosed. Thirteen patients (31%) died during treatment; from them, six were newly diagnosed. Median CSF white blood cell counts were significantly higher in nonsurvivors; the most common diagnosis was focal brain lesions; toxoplasmosis and tuberculosis were the first common etiologies; 79% recovered with the intended treatment regimen. History of drug abuse, not receiving antiretrovirals, low baseline CD4 counts, and loss of consciousness at the time of admission has been seen more among deceased patients. Conclusions: Neurologic presentations or complications of HIV infection lead to high mortality rates. Early diagnosis of infection and improvement of patient compliance with antiretroviral treatment can reduce the mortality associated with neurologic diseases.