Overall, patients with primary Sjögren's syndrome (pSS) in a Taiwainese nationwide cohort (n = 7,852) are at no greater risk of developing cancer than the general population. However, for the 25–44-year age group the incidence of cancer was increased, with a standardized incidence ratio of 2.19 (95% CI 1.43–3.21) compared to 1.04 (95% CI 0.91–1.18) for patients with pSS over all age groups.