Background: Lack of survival improvement in adolescents and young adults (AYA) with cancer has led to increased awareness of this young population. Design: We carried out a population-based study of incidence and survival of primary tumours and second primary tumours in patients aged 12–24 in north Netherlands. Age-specific incidence rates per 100 000 and 3-year moving means were calculated. Factors associated with incidence and survival were assessed using a Poisson model, log-rank test and multivariate Cox proportional hazards analysis. Results: From 1989 to 2003 a total of 1118 patients were diagnosed. The total age-specific incidence rates per 100 000 were as follows: males: 13.4 (12–15 years), 26.9 (16–19 years) and 27.5 (20–24 years) and females: 13.9, 20.7 and 20.7. Male : female ratio was 1.32. The overall estimated annual percentage change (EAPC) in incidence was 2.15% ( P < 0.01). Five-year survival was 80.8% and did not improve during the study period. With median follow-up of 5.5 years (range 0.0–16.0) in our cohort the standardized incidence ratio (SIR) of second primary tumours was 30.55 (95% confidence interval = 19.96–44.76, P < 0.05). Conclusions: The total incidence of cancer in AYA increased (EAPC = 2.15%). Survival was unchanged. The SIR of a second primary tumour in this young cohort increased 31-fold. Further research is needed to study this increasing incidence and optimise treatment outcome in these young patients.