Congenital asplenia has been traditionally described in association with cardiopulmonary anomalies (Ivemark syndrome). A case of congenital asplenia with duplication of the hindgut and genitourinary tract, myelomeningocele, situs inversus abdominis, and imperforate anus generated interest in review of the gastrointestinal anomalies of 36 patients who died of the disease in Childrens Hospital of Los Angeles. A significant number were found to have situs inversus, malrotation of the bowel, esophageal varices, duplication and hypoplasia of the stomach, Hirschsprung's disease, imperforate anus, and duplication of the hindgut. These patients present with symptoms referable to their gastrointestinal anomalies and may not draw attention to the concomitant cardiopulmonary anomalies or to the absence of the spleen. It becomes important, therefore, to establish the presence of the spleen in infants suffering from various anomalies of the gastrointestinal tract because of the overwhelming septicemia that is known to complicate the clinical management of these patients.