The Faroese population isolate harbors epidemiological and genetic characteristics that likely differ from outbred populations. This population-based register study found that the Faroese 2010–2020 crude incidence of amyotrophic lateral sclerosis (ALS) was 4.9/100,000 person-years (95% confidence interval [CI], 3.3–7.0) and the age- and sex-standardized incidence (US 2010 Census Population) was 4.1/100,000 person-years (95% CI, 2.7–6.0), which is a 68% increase from the 1987–2009 estimate. The 2020 crude prevalence was 9.5/100,000 (95% CI, 3.0–19.6) in a population of 52,912 inhabitants. Incidence and prevalence estimates of ALS in the Faroes are high and further research is warranted to uncover the genetic or environmental determinants of ALS in this population.