Background: Neonatal tetanus (NNT) is vaccine preventable disease. Study was conducted to provide magnitude and baseline information for evidence-based intervention. Methods and materials: A retrospective descriptive followed by case control study was conducted in district Naseerabad. Case was defined “any neonate resident of Naseerabad with a normal ability to suck and cry during the first 2 days of life; and who between 3-28 days of age cannot suck normally and becomes stiff or has convulsions during 2016–2019”. Hospital record and NNT surveillance data, case investigation form reviewed. Verbal autopsy and inspection of delivery scenes were conducted. The pre-tested questionnaire was used to collect demographic, clinical and risk factor information. Epi info version 7 was used for data compilation and analysis. Results: A total 138 cases identified. Mean age was 8.6 days with range from 4–22 days. Male female ratio was 1.5:1. Symptoms were fits 108 (78.3%), inability to suck 102 (73.9%), fever 91 (65.9%), locked jaw 77 (55.8%) and neck stiffness 57 (41.3%). Case fatality rate was 48.6%. Mean days from onset to fatality was 6 days. 123 (89%) deliveries were conducted at homes by untrained traditional birth attendant with zero TT shots. Survived had significantly greater mean bodyweight, later onset of disease, hospitalized early and received TIG while generalized rigidity, fever, fits, cyanosis, undercurrent infections and respiratory arrest were significantly more common in fatal group. Delivery scenes were unclean on inspection. Significant statistical associations were found between umbilical cord cutting with unsterilized scissor (OR 12.9; CI 1.4–13; p value <0.05) and NNT. Umbilical cord tied with unsterilized thread (P = 0.002), ash used to cover umbilical cord (P: 0.004), no hand washing during delivery by TBAs (P: 0.006), illiteracy (P: 0.0002), low socioeconomic status (P: 0.003), and nonuse of antenatal care services played aggravating role. Conclusion: Poor TT immunization and umbilical cord cutting with unsterilized scissor were probable cause. Lack of awareness regarding antenatal care and poor hygienic deliveries were main risk factors. Enhancing antenatal care, improvement in TT vaccination coverage and awareness in community were recommended.