We evaluated the clinical characteristics and outcome of tracheostomy in amyotrophic lateral sclerosis (ALS) using data from the Piemonte and Valle d'Aosta Register for ALS, a prospective epidemiological register collecting all ALS incident cases in two Italian regions. Among the 1260 patients incident in the period 1995-2004, 134 (10.6%) underwent tracheostomy. Young male patients were more likely to be tracheostomised. Site of onset (bulbar vs spinal) and period of diagnosis (1995-1999 vs 2000-2004) did not influence the likelihood of being tracheostomised. The mean duration of hospital stay was 52.0 days (SD 60.5). Overall, 27 patients died while still in hospital (20.1%). Sixty-five patients (48.5%) were discharged to home, whereas 42 (31.3%) were admitted to long-term care facilities. The median survival time after tracheostomy was 253 days. In the Cox multivariable model, the factors independently related to a longer survival were enteral nutrition, age, marital status and ALS centre follow-up. In conclusion, in an epidemiological setting, ALS survival after tracheostomy was <1 year. Sociocultural factors influence the probability of choice to be tracheostomised, even in a highly socialized health system as Italian one.