Background: Neonatal pneumothorax (NP) in neonates is a medical emergency with a significant incidence of morbidity and mortality. There is a paucity of national and regional data about the epidemiological and clinical profiles of pneumothorax.Aim: The study aim is to identify the demographics, predisposing factors, clinical profiles, and outcomes of NP in a tertiary neonatal care center in Saudi Arabia.Methods: A retrospective study of all newborns admitted at the neonatal intensive care unit at International Medical Centre, Jeddah, Saudi Arabia, over seven years period between January 2014 and December 2020 was reviewed. A total of 3,629 newborns admitted to the neonatal intensive care unit were included in the study. Data collected included baseline characteristics, predisposing factors, associated morbidities, management, and outcomes of NP. Data were analyzed using the Statistical Package for Social Sciences (SPSS) version 26 (IBM Corp., Armonk, NY).Results: Of a total of 3,692 included neonates, pneumothorax was detected in 32 neonates with an incidence of 1.02% (ranging from 0.69% to 2%), and 53.1% were males. The mean gestational age was 32 weeks. Our study found that most infants with pneumothorax were extremely low birth weight (ELBW) in 19 babies (59%). The most common predisposing factors were respiratory distress syndrome in 31 babies (96.9%) followed by the need for bag-mask ventilation in 26 babies (81.3%). Twelve newborns (37.5%) with pneumothorax died. Following an analysis of all risk variables, the one-minute Apgar score <5, associated intraventricular hemorrhage, and respiratory support need were shown to be significantly linked with death.Conclusion: Pneumothorax is not an uncommon neonatal emergency event, especially for ELBW infants, infants requiring respiratory support, or infants with underlying lung disease. Our study describes the clinical profile and affirms the significant burden of NP.