Segmental dilatation of ileum (SDI) is a rare clinical entity and so is anterior thoracic meningomyelocele (AMC). There has been no reported association between these two clinical entities. We hereby report a very rare presentation of these two in a 4 year old boy who presented with swelling in the right lower abdomen. Preoperative diagnoses were partial cecal volvulus and duplication cyst. At operation, SDI along with AMC was found.