Although the prevalence rates of celiac disease tend to be very similar in different Western populations, mortality rates for this disease vary widely. In this Review we focus on the papers that have addressed this issue so far. We evaluated mortality rates in different forms of celiac disease, such as symptomatic celiac disease, unrecognized celiac disease, dermatitis herpetiformis and refractory celiac disease. We also evaluated the role of possible protective factors, such as adherence to a gluten-free diet, early diagnosis and severity of clinical presentation. Finally, we noticed that the mortality rate for celiac disease seems to be higher in Southern than in Northern Europe and seems to correlate with 'national' gluten consumption. To explain these differences, we propose a hypothesis that links mortality rates to the amount of gluten consumed not only after but also before the diagnosis of celiac disease.