We report on a patient whose neurological and neurophysiological findings fulfil the El Escorial criteria for definite amyotrophic lateral sclerosis (ALS), and who is still alive 44 years after the initial diagnosis. Pertinent differential diagnoses were excluded on clinical and/or genetic grounds. Our patient has no afflicted relatives and her SOD1 testing was negative, thus allowing us to classify her form of ALS as sporadic. Informing ALS patients of the existence of documented cases with long-term survival can be a means of fostering hope when delivering the diagnosis.