Although the median survival in polycythemia vera (PV) is 14 years, mortality is higher than in an age- and sex-matched population. This study included 3941 PV patients diagnosed between 2000-2012 from Surveillance, Epidemiology and End Results (SEER) 13 registry to determine 5-year survival and the incidence of second primary malignancies (SPM). The actuarial 5 year survival in the overall cohort was 79.5%. The cumulative incidence of SPM was 13.1% at 10 years. SPMs occurred at a standardized incidence ratio (SIR) of 1.29 (95% CI = 1.16-1.43; p < 0.001) with an absolute excess risk (AER) of 42.49 per 10 000 population. A significantly higher risk was noted for acute myeloid leukemia (SIR = 12.24; 95% CI = 8.17-17.8; p-value < 0.001) and chronic myeloid leukemia (SIR = 10.66; 95% CI = 3.75-19.6; p-value < 0.001). Patients with PV are at a high risk of SPM and leukemic transformation, which may compromise long-term survival.