Heterotopic salivary glandular tissue within the rectum is exceptionally rare. To date, only 4 cases have been reported in literature and all in adult patients. Here we report a case of ectopic salivary glandular tissue in the rectum in a 12-year-old girl who also had a lumbosacral lipomeningocele. The coexistence of these 2 developmental anomalies; 1 endodermal and 1 neuroectodermal, has not been reported previously. The case is discussed with emphasis on embryologic correlation, review of literature, and a summary of prior published cases.
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