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Introduction: The prevalence and incidence of amyotrophic lateral sclerosis (ALS) is not fully established, and this varies depending on the studied population. Objective: To estimate the prevalence/incidence of ALS patients in Antioquia-Colombia. Methodology: Observational/descriptive study by reviewing clinical records from 2010 to 2014. Cases with possible, probable and definite ALS were included. To estimate the prevalence/incidence, capture-recapture method was used. Results: Point prevalence in December 2014 was 4.9/100,000 (95% CI 2.0–7.8), and the incidence was 1.4/100,000/year (95% CI 0.5–2.2). The median survival was 4 years. Spinal-onset was observed in 62.4% of the included patients. Conclusion: Prevalence, incidence and clinical presentation of ALS in Antioquia are similar to most studies reported worldwide. However, prevalence in Antioquia seems to be slightly higher than in other studies from Latin -American countries. This may derive from the inclusion criteria and case detection methodology adopted, but sociodemographic and genetic factors should be considered. [ABSTRACT FROM AUTHOR]