Two cases of pediatric blue rubber bleb nevus syndrome are reported. The main features of the disease are rubbery blue cutaneous nevi and hemangiomatous, frequently hemorrhagic malformations of the bowel wall. Polypoid filling defects were seen throughout the bowel on barium studies. This entity should be considered in the differential diagnosis of polyposis, and the skin should be carefully examined for nevi when multiple bowel polyps are demonstrated, especially in patients with gastrointestinal bleeding.