Background: Intestinal atresia is a very common cause of neonatal intestinal obstruction. Jejunoileum is the commonest site of intestinal atresia. The frequencies of Jejunoileal atresia are still few in developing countries. We have not enough research-based information regarding the presentation and outcomes of jejunoileal atresia treatment. Aim of the study: The aim of this study was to assess the presentation and outcomes of neonates with jejunoileal atresia. Methods: This prospective observational study was conducted in the Department of Pediatric Surgery, Bangladesh Shishu Hospital, and Institute, Dhaka, Bangladesh during period from July 2016 to June 2020-. In total 27 neonates with jejunoileal atresia were included as the study subjects for this study. Patients with suspected jejunoileal atresia (JA) but who died before surgery and duodenal or colonic atresia were excluded from the study. A predesigned questionnaire was used in data collection. All data were collected, processed, and analyzed by using MS Office and SPSS version 23.0 programs as per need. Results: In this study, the male-female ratio was 1:1.25. The highest number of participants were with Type I atresia which was 37%. In the majority of the participants (59%) as a primary surgical procedure, a single small bowel anastomosis was performed. As early complications ‘Clavien-Dindo ≥III’ and cholestasis were found among 26% and 37% of patients respectively which were considered the most frequent. On the other hand, as of late complications, short bowel syndrome was found in 22% of patients which was noticeable. Finally, in this intervention 4% cases of mortality were found whereas 96% were survived. Conclusion: Till now intestinal atresia (IA), as well as jejunoileal atresia, are associated with unacceptably high morbidity as well as mortality because of inadequate neonatal intensive care, late presentation, and lack of parenteral nutritional support, especially in the countries of the third world.