We present a low-birth weight newborn who presented with bilious vomit and had a "double bubble" sign on plain abdominal X-ray. Intraoperatively, we found annular pancreas with malrotation and type I choledochal cyst. To the best of our knowledge, this is the first reported case with a combination of these anomalies.
Keywords: Annular pancreas; malrotation; type I choledochal cyst.