Abstract Background Intestinal obstruction in children may be congenital, acquired, intrinsic or extrinsic. Most intestinal obstructions in children are the result of postoperative adhesions. Those caused by anomalous congenital band are extremely rare. Clinical case Patient of a 1-year-old male, with no previous history of abdominal surgery or trauma. He suffered with vomiting of bile content and loss of appetite at home three days before admission. On physical examination, he was irritable, with abdominal distention, absence of peristalsis and abdominal tenderness, with no signs of peritoneal irritation being found. The blood count reported leucocytosis and the x-rays show dilated small bowel loops, fluid levels and absence of air in rectal ampulla. An open laparotomy was subsequently performed, and the intraoperative findings were consistent with a congenital band extending from the anti-mesenteric wall of the jejunum to the root of mesentery, compressing the ileum at 50 cm from the ileocecal valve, causing ischaemia. The band was ligated and divided, with an uneventful postoperative course. He was discharged 4 days later without complications. Conclusions The anomalous congenital band is not associated with abdominal problems, such as remnants of previous laparotomies or embryological structures, such as vitelline vessels or omphalomesenteric yolk duct. These bands seem to have a congenital origin and cause bowel obstruction by trapping between the band and the mesentery. The diagnosis of anomalous congenital band is extremely difficult and no imaging study is useful for the diagnosis, and only exploratory laparotomy or laparoscopy is useful for diagnosis and treatment of this problem.