Background Esophageal perforation is a rare and life-threatening condition with several treatment options. The aim was to assess the incidence, type of treatment and mortality of esophageal perforations in Sweden and to identify risk factors for 90-day mortality. Method All patients admitted with an esophageal perforation from 2007 to 2017 were identified from the National Patient Register. Mortality was assessed by linkage with the Cause of Death Registry. We analyze the incidence and the impact of age, sex, comorbidities on mortality. Results 879 patients with esophageal perforation were identified, giving an incidence rate of 1.09 per 100,000 person-years. The median age at diagnosis was 68.8 years and 60% were men. The mortality was 26% at 90 days. Independent risk factors for death within 90 days were age (odds ratio (OR): 6.20; 95% (confidence interval) CI: 2.16-17.79 at 60-74 years and OR: 11.58; 95% CI: 4.04-33.15 at 75 years or older), peripheral vascular disease (OR: 2.92; 95% CI: 1.44-5.92) and underlying malignant disease (OR: 5.91; 95% CI: 3.86-9.03). In patients younger than 45 years, survival was lower among women than among men (at 5 years 73 and 93%, respectively). The cause of death among young women was often drug-related or suicide. Conclusions 90-day mortality was 26%, old age, vascular disease and underlying malignant disease were risk factors.