Background: This study was done at SMS Medical College, Jaipur with an aim of emphasizing the need of having a high index of suspicion in newborn presenting with bilious vomiting for the diagnosis of malrotation. Methods: Cases of neonatal small intestinal obstruction due to malrotation presenting to the Department of Paediatric Surgery, SMS Medical College, Jaipur over a period of two years were evaluated. Results: A total of 48 newborns presented to our department with intestinal obstruction due to malrotation. Majority (82%) of the patients presented in the 2nd and 3rd week of life. 6 out of 48 patients presented with extensive gangrene of midgut. 9/48 patients died during the course of treatment and during follow-up. Conclusion: Malrotation is a relatively common cause of neonatal small bowel obstruction. A high index of suspicion is required in neonates presenting with bilious vomiting. Prompt diagnostic tools and early laparotomy prevents fatal complication of extensive midgut gangrene due to volvulus.