Celiac disease (CD) is a chronic autoimmune condition affecting 1% of genetically predisposed individuals by inducing CD-specific antibodies and enteropathy and has been linked to an increased risk of both intestinal and extraintestinal malignancies. The pathogenesis of CD is not completely understood, yet environmental factors are at the core of disease onset and progression, with gluten acting as the sine qua non trigger. The most common types of tumors found in CD patients are lymphomas and adenocarcinomas. A subset of patients having type 2 refractory CD develop enteropathy-associated T cell lymphoma. Surveillance of CD patients and close monitoring of strict adherence to the gluten-free diet have shown to reduce the rate of cancer occurrence associated with CD. This chapter highlights the epidemiology, presentation and the pathogenic mechanisms implicated in most common types of cancers associated with CD.