Intestinal atresia accounts for about one third of all cases of neonatal intestinal obstruction. The survival rate has improved to 90% in most of the series with the operative mortality being < 1%. The survival rate improves with distal atresias. An increased mortality is observed in multiple atresias (57%), apple peel atresia (71%), and when atresia is associated with meconium ileus (65%), meconium peritonitis (50%) and gastroschisis (66%). Although appearance of echogenic bowel on prenatal ultrasonography is suggestive of GI, it is confirmed in only 27% cases. Prenatal ultrasonography is more reliable in detection of duodenal atresia than more distal lesions. Short bowel syndrome is the major impediment in the management of jejunoileal atresia. Although total parenteral nutrition (TPN) is the main adjunctive treatment, it delays intestinal adaptation and may cause cholestasia and subsequent liver damage. Graduated enteric feedings, use of growth hormone, glutamine and modified diets containing low fat, complex carbohydrates and protein supplements have been used in a adults with short bowel syndrome to successfully diminish TPN requirements and enhance nutrient absorption in nearly half of the patients. Utilization of growth factors to facilitate intestinal adaptation and advances in small bowel transplant may improve the long-term outcomes in future.