Summary Background Liver cirrhosis is characterized by a silent phase until decompensation, which is defined by ascites, bleeding from esophageal varices or hepatic encephalopathy. Aim To compare the clinical course, patterns of survival and causes of death by etiology during long‐term follow‐up in a large population‐based cohort of patients with incident cirrhosis. Methods We used population‐based medical registries for a cohort study of patients with liver cirrhosis diagnosed January 2001 to December 2010, in the Scania region of Sweden. Medical records were reviewed. Patients were classified according to etiology and clinical parameters were registered. Patients were followed until December 2017. Results The cohort comprised 1317 patients, 631 were decompensated at diagnosis and 387 decompensated during follow‐up. The cumulative 10‐year incidence of decompensation, with death and transplantation as competing risks, was 89% in alcoholic cirrhosis, 58% in hepatitis C and 75% in cryptogenic cirrhosis. The lowest 10‐year transplantation‐free survival rates were found in cryptogenic cirrhosis (11%), alcohol‐related cirrhosis (18%) and alcohol combined with hepatitis C (12%). Autoimmune hepatitis cirrhosis showed the best 10‐year survival (53%) and hepatitis C, non‐alcoholic steatohepatitis, primary biliary cholangitis, and primary sclerosing cholangitis and other causes averaged 30%. Decompensation at diagnosis was an important predictor for death in all etiologies apart from alcoholic cirrhosis. 991 patients died and 91 were transplanted. Conclusion Our results show that the clinical course and survival in cirrhosis differ considerably by both etiology and state at diagnosis.