Celiac disease is an autoimmune enteropathy developing in genetically predisposed individuals after mucosal contact with gluten, secondary to unknown triggering factors. An increased rate of malignancies in adults with celiac disease has been confirmed in several studies, but there is considerable evidence that a gluten-free diet protects against the development of cancer. The association between celiac disease and childhood cancer is not clear. Few cases are reported in the literature. Here, we report the cases referred to our pediatric oncology unit with a review of the literature.