Neonatal tetanus (NNT) is still one of the major preventable causes of neonatal death in Nigeria. It is a disease of poverty, adverse social and environmental conditions. The aim of the study was to review neonatal tetanus cases, determine the prevalence, disease outcome and what possible interventions can be done in the study area to reduce its prevalence. This was a retrospective study of cases of NNT seen in Special Care Baby Unit of Ahmadu Bello University Teaching Hospital, Zaria, Nigeria from 2001 to 2014. Case notes of neonates with clinical diagnosis of tetanus who were admitted into the unit were retrieved for analysis. Data extracted included: socio-demographic characteristics, antenatal clinic (ANC) history, TT immunization, place of delivery and disease outcome. There were a total of 60 cases of NNT during the period; this gave an annual prevalence of 4.3 per year. Mean age of neonates was 9.3 ± 5.1 days, M: F ratio was 4.0:1.0. Mean age of mothers was 23.2 ± 4.0 years, majority (55%) had no formal education, 48% had ≤ 2 ANC visits, and more than 70% had no TT immunization and delivered at home. Commonest probable portal of entry of infection was umbilicus (70%) and major presenting symptoms/signs were: spasms (81.7%), poor suckling (81.7%), inability to open mouth (45%) and fever 45%, respectively. Site of infection and presence of fever were associated poor outcome. Mortality among neonates who had short incubation period (≤ 6 days), umbilicus as probable site of infection (P=0.006) and presence of fever (P=0.014) were significantly higher for non-survivors than survivors. Overall, case fatality rate (CFR) was 56.7%. The review revealed that CFR is still unacceptably high for a disease that can be prevented and eventually eliminated with cost effective and affordable public health interventions. All the 3 tiers of government need to re-focus the National Immunization Policy together with sustained immunization programmes throughout all the communities in Nigeria. There is urgent need for health education at community level on the importance of ANC and mass immunization regardless of age in order to achieve the goal of NNT elimination.