celiac disease (cD) is a chronic enteropathy caused by the ingestion of gluten in genetically susceptible individuals. cD is a common food-related disorder with a prevalence of ~1% worldwide. Failure to follow the only available treatment, i.e., a standard gluten-free diet (GFD), increases the risk of adverse outcomes, such as refractory cD. Studies have reported that the long-term avoidance of a GFD may lead to the development of certain types of cancer in patients with cD. An increased risk of gastrointestinal cancers and intestinal lymphomas are associated with cD. On the other hand, recent studies have demonstrated that the risk of colon cancer, ovarian and breast cancer is low in patients with cD. It has also been demonstrated that a strict GFD exerts a positive effect in reducing the cancer risk. However, only a limited number of studies have been conducted in this area, and the outcomes of these studies warrant further verifications. The present review article summarises and discusses the possible links between cD and cancer, and the probable reasons behind their association. In addition, the present review also discusses whether a strict GFD reduces the risk of developing certain types of cancer in patients with cD.