Background: To explore trends in the incidence and survival of patients with intrahepatic cholangiocarcinoma (ICC) an unselected population in Western Europe was studied. Methods: Between 1989 and 2009, all patients newly diagnosed with ICC were selected from the Netherlands Cancer Registry (n= 809). Trends in incidence, treatment and relative survival were calculated according to gender and age. Follow-up for vital status was complete until 1st January 2010. Results: The incidence rates of ICC increased significantly between 1999 and 2009, especially in the age group 4559 years [estimated annual percentage change +3.0%, 95% confidence interval (CI) 0.25.8]. In the other age groups ICC incidence remained stable. Patients diagnosed with Tumour Lymph Node Metastasis (TNM) stage I mainly underwent surgery (68%), and the majority of the patients with stage II, III and IV received best supportive care (73%). One-year relative survival for patients with ICC increased significantly from 24% in 19891994 to 28% in 20052009 (P= 0.03), and corresponding 3-year relative survival improved from 4% to 8% (P= 0.02). Three-month and 1-year relative survival for patients with ICC receiving surgery was 91% and 71%, respectively. Discussion: Between 1999 and 2009, the incidence of ICC rose, especially in the age group 4559 years, suggesting aetiological influences. Survival rates have improved during the study period.