Tumors of the pediatric gastrointestinal tract are extremely rare. Their infrequent presentation at treatment centers has not allowed for the development of standardized treatment protocols and prospective review. The most prevalent gastrointestinal neoplasms and malignancies are described, including gastrointestinal lymphoma, colorectal carcinoma, carcinoid tumors, gastrointestinal stromal tumors, leiomyomas, juvenile polyps, inflammatory pseudotumors, gastric tumors, and Peutz-Jeghers polyposis syndrome. Current recommendations for the medical and surgical management of these tumors are reviewed and summarized for this vast group of gastrointestinal neoplasms in children.