Understanding how health outcomes differ for patients with advanced cystic fibrosis (CF) lung disease living in the United States compared with Canada has health policy implications. What are rates of lung transplant (LTx) and rates of death without LTx in the United States and Canada among individuals with FEV1< 40%predicted? This was a retrospective population-based cohort study, 2005 to 2016, using the US CF Foundation, United Network for Organ Sharing, and Canadian CF registries. Individuals with CF and at least two FEV1 measurements< 40%predicted within a 5-year period, age≥ 6 years, without prior LTx were included. Multivariable competing risk regression for time to death without LTx (LTx as a competing risk) and time to LTx (death as a competing risk) was performed. There were 5,899 patients (53%male) and 905 patients (54%male) with CF with FEV1< 40%predicted living in the United States and Canada, respectively. Multivariable competing risk regression models identified an increased risk of death without LTx (hazard ratio [HR], 1.79; 95%CI, 1.52-2.1) and decreased LTx (HR, 0.66; 95%CI, 0.58-0.74) among individuals in the United States compared with Canada. More pronounced differences were seen in the patients in the United States with Medicaid/Medicare insurance compared with Canadians (multivariable HR for death without LTx, 2.24 [95%CI, 1.89-2.64]; multivariable HR for LTx, 0.54 [95%CI, 0.47-0.61]). Patients of nonwhite race were also disadvantaged (multivariable HR for death without LTx, 1.56 [95%CI, 1.32-1.84]; multivariable HR for LTx, 0.47 [95%CI, 0.36-0.62]). There are lower rates of LTx and an increased risk of death without LTx for US patients with CF with FEV1< 40%predicted compared with Canadian patients. Findings are more striking among US patients with CF with Medicaid/Medicare health insurance, and nonwhite patients in both countries, raising concerns about underuse of LTx among vulnerable populations.