Although the absolute risk of enteropathy-associated malignancies in celiac disease is generally very small due to low prevalence/incidence rates, the relative risk may be considerable so that prevention strategies based on appropriate data seem clinically desirable. The great majority of the case-control and cohort studies which have been published in the last years point to a significantly elevated risk for tumors in the gastrointestinal (GI) tract in terms of hazard/odds ratios, observed/expected ratios, and/or standardized incidence/morbidity ratios in the magnitude>5.0, whereas the risk for tumor outside the GI tract seems to be much lower (PubMed December 2007). Chronic inflammation with persistent symptoms/complaints and especially chronic refractory disease type II are considered to be particular individual risk factors. In addition, genetic factors and/or certain gene combinations may unfavorably influence the course of the disease. In the absence of controlled and prospective trials and corresponding evidence-based guidelines as they have been published for colon cancer, the strategies of prevention in patients with celiac disease differ from center to center according to their own experience/ expertise. Besides regular clinical, serologic/immunologic and endoscopic/histologic assessment a careful history taking with special regard to the disease course and a detailed ultrasound examination of the entire abdomen in patients at risk may play an important role in the long-term follow-up.