Although tetanus is a preventable disease by vaccination, it continues to claim lives around the world. Whereas cases of accidental origin reflect insufficient population immunization, tetanus neonatorum reveals a double-nature fault-poor vaccination coverage of adults coupled with difficulties accessing appropriate prenatal care; this situation is aggravated by the extreme severity of tetanus in this age group in which the mortality rate can reach up to 80%. The early detection of tetanus in neonates is essential for immediately initiating the proper therapy. Therefore, although reaching an early diagnosis of tetanus is important, the most relevant aspect is related to the appropriate management and prophylaxis of this disease. Consequently, the aim of this article is to review neonatorum tetanus with an emphasis on its therapy and prevention.