While military service is an established risk factor for amyotrophic lateral sclerosis (ALS), it remains unclear whether this association is linked to combat. We conducted a matched case-control study comparing 191 ALS patients who were veterans of the Israeli Defense Forces (IDF) with known military service type and 1910 matched controls. The ALS group had higher rates of combat service (46.0% vs. 22.7%) and parachuting (10.5% vs. 1.1%) in comparison with controls (p < 0.001 for both). In a multivariate model, combat service was associated with ALS (odds ratio 2.49, confidence interval [1.49-4.16], p < 0.01). The higher prevalence of combat roles among ALS patients expands our understanding of military service factors that contribute to ALS risk.