The annular pancreas is represented by a complete or incomplete ring of pancreatic tissue surrounding the descending part of the duodenum. This defect is formed at 5—6 weeks of the embryonic period due to abnormal rotation of the pancreatic buds around the duodenum. In most clinical cases, the annular pancreas is manifested in the neonatal period with symptoms of complete high intestinal obstruction. In rare cases, it is the cause of chronic duodenal obstruction in adult patients. The annular pancreas is a complex anomaly of the pancreatobiliary zone and is often accompanied by pancreatobiliary maljunction; it causes the development of pancreatitis, impaired passage of bile in adulthood. The article presents the clinical observation of a 10-year-old child with decompensated duodenal obstruction on the basis of the annular pancreas with the development of cystic dilation of the common bile duct. The patient received laparoscopic extrahepatic bile duct resection, cholecystectomy, antrumectomy, Roux-en-Y hepaticojejunostomy and gastrojejunostomy. A brief review of the literature is provided.