Tumors of the gastrointestinal (GI) tract are rare in children with less than 5% of childhood tumors arising from the GI tract [1]. Due to non-specific presentation and low degree of clinical suspicion, the diagnosis is often delayed. The most common GI tumors of childhood are lymphoma, gastrointestinal stromal tumor (GIST), carcinoma, carcinoid, and other benign tumors [2]. Both malignant and benign tumors of GIT in the pediatric age group may have an underlying genetic disorder, and it is essential for a radiologists to be familiar with the imaging findings as well as the associations.