Atresia of the distal ileum concurrent with ileocaecal valve agenesis is one of the rare types of intestinal atresia with very few reports in the literature. We herein present a case of type II distal ileal atresia, ileocaecal valve agenesis and volvulus of the ileum and the terminal jejunum with a history of depression, anorexia and failure to pass meconium. On necropsy, volvulus of the ileum and the terminal jejunum was found. The ileum was distended, full of fluid and gas, and similar to a blind-end sac. The ileocaecal valve was absent and the ileum was attached to the caecum by a fibrous band. The caecum was atrophied and small. The distal segments of the intestine were smaller than normal. These findings revealed type II terminal ileal atresia simultaneously agenesis of the ileocaecal valve and intestinal volvulus probably secondary to atresia in a newly born goat kid.