Aims: This paper aims to investigate a case of neonatal tetanus in Saudi Arabia, studying the different factors leading to infection, role and protocols used in hospital in diagnosis and management of the case. Methods: A medical profile of neonatal case confirmed for neonatal tetanus was collected and studied, in addition to collecting literature reviews about epidemiology, diagnosis and management. Results: A newborn 7-day old girl with poor socioeconomical status and poor aseptic delivery had been admitted to hospital with symptoms of jerky repetitive movement and history of decreased feeding and poor sucking for one day. Laboratory tests, cranial computed tomography (CT) and lumbar puncture were performed, all of them with normal results. She was hospitalized on 12 November, 2019 in an environment free of sensorial stimuli in the Pediatric Intensive Care Unit (PICU), with assisted ventilation. The patient was treated with metronidazole 30mg/kg/day, ampicillin 150 mg/kg/day, and cefotaxime 150mg/kg/day. Sedation was maintained with midazolam 6mic/kg/min. Also, we started on muscle relaxation pancuronium 0.1 mg /kg/hr. and Magnesium sulfate. Besides that, we administered human immunoglobulin IM (500 IU), as well as one dose of tetanus toxoid. During hospital admission the condition of the patient was much improved. On 19 December 2019 the patient was extubated and started with baclofen and physiotherapy. Conclusion: This case indicates that non-sterile delivery by non-practical attendants are the biggest cause of neonatal tetanus along with mothers unimmunized against tetanus. However, in the rarest of incidence of neonatal tetanus, physicians should be prepared to suspect, diagnose and treat neonatal tetanus and ensure clear and clean delivery of newborns. A combination of antibiotics and muscular relaxants are used to manage neonatal tetanus. Key words: neonatal tetanus, case report, Saudi Arabia