IMPORTANCE Orofacial cleft (OFC) is one of the most common congenital malformations, with a wide variation in incidence worldwide. However, population-based studies on the incidence of OFC in North America are lacking. OBJECTIVES To examine the incidence of OFC in Ontario, Canada, and to compare risk factors and mortality associated with children with OFC vs children without OFC. DESIGN, SETTING, AND PARTICIPANTS This population-based retrospective cohort study used health administrative data from the province of Ontario, Canada. Children with OFC who were born from April 1, 1994, to March 31, 2017, in Ontario were each matched to 5 children without OFC based on sex, date of birth (±30 days), and mother's age (±5 years). Analyses were conducted from September 2018 to January 2019. EXPOSURES Children born with OFC. MAIN OUTCOMES AND MEASURES Incidence of OFC over time and regional variation. Risk factors for OFC were assessed using 1-way analysis of variance for means, Kruskal-Wallis for medians, and χ 2 tests for categorical variables. Adjusted Cox regression models were used to assess mortality.