Aim To assess the incidence and mode of presentation of type 1 diabetes mellitus (T1DM) in children and adolescents younger than 14 yr of age between January 2006 and December 2010 in Malta. Methods A nationwide prospective study which collected data from newly diagnosed T1DM children who presented to the only paediatric diabetes team available in Malta. The degree of ascertainment was estimated to be 100%. Incidence rates by age group and year were estimated using real values of diagnosed patients and population statistics. Trend analysis was carried out using Poisson's regression analysis. Results From 2006 to 2010, 81 children below the age of 14 yr were diagnosed with T1DM for the first time. The age- and sex-standardised incidence rate was 21.86/100,000 children/yr. The estimated annual increase in incidence was 21.8%. Compared to data collected retrospectively between 19962001, the incidence has increased threefold between 2006 and 2010. Generally, the incidence rate was highest in the 59 yr age group, followed by the 04 yr age group and finally the1014 yr age group. However, the highest annual increase occurred in the 04 yr age group at 39% per year, closely followed by 59 yr age group at 31% per year. In the 1014 yr age group, the trend appeared to show a reduction in incidence. The proportion of patients presenting in diabetic ketoacidosis (DKA) was high at 41%. Conclusion In Malta, the number of children/adolescents with T1DM has been rising at a faster rate than expected, and a distinct shift to younger age at onset has been observed. DKA rate at presentation is still high in Maltese children.