Introduction: Neonatal tetanus is a highly debilitating disease with high mortality. Global efforts at eliminating the disease in developing countries are yielding results but slower than expected. The high case fatality of neonatal tetanus remains a therapeutic challenge to physicians and requires continuous evaluation. This study aimed to determine the incidence and outcome of neonatal tetanus in Zaria, northwestern Nigeria. Methodology: A retrospective study of neonatal tetanus was conducted at the Neonatal Unit of Ahmadu Bello University Teaching Hospital, Zaria, between January 2005 and December 2009. Data generated were analyzed using the Epi Info version 3.5.1 software and statistical significance was set at p < 0.05. Results: Of the 2,692 newborns admitted during the study period, 20 had tetanus, giving an overall prevalence of 0.7%. There were 14 males and 6 females (male to female ratio 2.3:1). The mean age and weight at presentation were 8.3 ± 4.0 days and 2.7 ± 0.3 kg, respectively. The mean incubation period was 3.2 ± 2.0 days. Four infants (20.0%) survived, one was discharged against medical advice and 15 (75.0%) died. Factors associated with mortality were presentation at less than seven days of life, low Hendrickse score at presentation (p = 0.0005) and hypoglycaemia (p = 0.0374). Conclusion: The incidence and the mortality rate of neonatal tetanus appear to be lower than those previously reported by our centre for the same region. Therefore, the ongoing global efforts for disease elimination and further improvements in the quality of care should be sustained.