Context:Jejunoileal atresia (JIA) is a common abnormality. The outcome is conditioned by several variables. Nutritional problems, and long-term sequelae are described among those who survive.Aim:To correlate the type of JIA and its management to the outcome and long-term quality of life.Settings and Design:Forty-three cases over a 17-year period (1992–2009). Perinatal data, management, and outcome were extracted from the clinical notes. The cases that had survived were contacted to get information about their present condition.Materials and Methods:Morbidity and mortality were matched to maturity, birth weight, mode of diagnosis, type of JIA, associated anomalies, and management and duration of parenteral nutrition. Growth and quality of life in 34 cases were evaluated via a telephone interview at a minimum of one year from surgery.Statistical analysis:Fisher test, Linear regression test, Kruskal-Wallis test, Dunn's comparison test.Results:Male/Female ratio was 25/18 and median birth weight was 2.644 g. Prenatal diagnosis was recorded in 34%. Six patients (14%) had associated anomalies. Primary surgery was resection and anastomosis in 88% and temporary stoma in 12%. Length of the resected bowel ranged from 3 to 65 cm. Whenever multiple atresia was found, the bowel length was saved by multiple anastomosis. Three dehiscences and three adhesive obstructions required a reoperation. Two patients (4%) died due to a central catheter–related sepsis. Prenatal diagnosis did not influence the outcome and was associated with a higher rate of Cesarean deliveries. Interview, at a median of nine years, showed normal growth in 85%. One case of short bowel syndrome is still on parenteral support at 22 months.Conclusions:Preserving bowel length and reducing the recourse to stoma is the key to good outcome and growth. Sequelae are correlated with the type of atresia and length of residual bowel; however, they decrease their severity with time.