BACKGROUND: No previous correlation has been performed of phenotype at diagnosis of Crohn's disease (CD) patients and mortality. We assessed the predictive value of phenotype at diagnosis on overall and disease related mortality in a European cohort of CD patients. METHODS: Overall and disease related mortality were recorded ten years after diagnosis in a prospectively assembled, uniformly diagnosed European population based inception cohort of 380 CD patients diagnosed between 1991 and 1993. Standardized Mortality Ratios (SMRs) were calculated for geographic and phenotypic subgroups at diagnosis. RESULTS: Thirty-seven deaths were observed in the entire cohort, whereas 21.5 deaths were expected (SMR 1.85 , 95 % CI: 1.30-2.55). Mortality risk was significantly increased in both females (SMR 1.93, 95% CI: 1.10-3.14) and males (SMR 1.79, 95% CI: 1.11-2.73). Patients from North European centres had a significant overall increased mortality risk (SMR 2.04, 95% CI: 1.32- 3.01), whereas a tendency towards increased overall mortality risk was also observed in the South (SMR 1.55, 95% CI: 0.80-2.70). Mortality risk was increased in patients with colonic disease location and with inflammatory disease behaviour at diagnosis. Mortality risk was also increased in the age group above 40 years at diagnosis for both total and CD related causes. Excess mortality was mainly due to gastrointestinal causes that were related to CD. CONCLUSIONS: This European multinational population-based study revealed an increased overall mortality risk in CD patients ten years after diagnosis and age above 40 years at diagnosis to be the sole factor associated with increased mortality risk.