Objective:To determine whether race/ethnicity and socioeconomic status are associated with amyotrophic lateral sclerosis (ALS) mortality in the United States.Methods:The National Longitudinal Mortality Study (NLMS), a United States–representative, multistage sample, collected race/ethnicity and socioeconomic data prospectively. Mortality information was obtained by matching NLMS records to the National Death Index (1979–2011). More than 2 million persons (n = 1,145,368 women, n = 1,011,172 men) were included, with 33,024,881 person-years of follow-up (1,299 ALS deaths , response rate 96%). Race/ethnicity was by self-report in 4 categories. Hazard ratios (HRs) for ALS mortality were calculated for race/ethnicity and socioeconomic status separately and in mutually adjusted models.Results:Minority vs white race/ethnicity predicted lower ALS mortality in models adjusted for socioeconomic status, type of health insurance, and birthplace (non-Hispanic black, HR 0.61, 95% confidence interval [CI] 0.48–0.78; Hispanic, HR 0.64, 95% CI 0.46–0.88; other races, non-Hispanic, HR 0.52, 95% CI 0.31–0.86). Higher educational attainment compared with < high school was in general associated with higher rate of ALS (high school, HR 1.23, 95% CI 1.07–1.42; some college, HR 1.24, 95% CI 1.04–1.48; college, HR 1.10, 95% CI 0.90–1.36; postgraduate, HR 1.31, 95% CI 1.06–1.62). Income, household poverty, and home ownership were not associated with ALS after adjustment for race/ethnicity. Rates did not differ by sex.Conclusion:Higher rate of ALS among whites vs non-Hispanic blacks, Hispanics, and non-Hispanic other races was not accounted for by multiple measures of socioeconomic status, birthplace, or type of health insurance. Higher rate of ALS among whites likely reflects actual higher risk of ALS rather than ascertainment bias or effects of socioeconomic status on ALS risk.