Leveraging genetic data from 11,130 people with ALS and 7416 controls from both the literature and Project MinE, we found that rare variants in the NEFH gene increase the risk of ALS (Project MinE p = 0.0007 and meta‐analysis p < 0.0001) and that this association was driven by missense variants in the tail domain and intronic variants in the rod domain. The gene discussed is NEFH; the disease is amyotrophic lateral sclerosis.