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Backgorund: Data on epidemiology and disease characteristics of amyotrophic lateral sclerosis (ALS) are geographically limited and no systematically collected data exist for slovenian patients. We performed a retrospective descriptive study on clinical attributes and disease course of patients with ALS treated at the Institute of clinical neurophysiology (ICN), University Medical Centre Ljubljana since the foundation of a specialised ALS group.Methods:  All 271 patients treated at ICN in the 10-year period between 2003 and 2012 were analysed. Data on basic demographic characteristics, phenotype of disease onset, diagnostic delay, survival, family history, use of percutaneous gastrostomy (PEG), of non-invasive ventilation and of riluzole were obtained.Results:  Mean age at symptoms onset was 62.7 ± 11.4 years, median diagnostic delay 11 (IQ range  7–19) months and mean survival from time of enrolment 16.4 ± 15.1 months.  179 (66.1%)patients had spinal onset disease and 71 (26.2%) bulbar onset disease. Factors associated with longer survival were lower age at enrolment, longer diagnostic delay and use of PEG. The proportion of patients using non-invasive ventilatory support was rising through the analysed years.Conclusions: Disease characteristics and survival in our series are similar to data from other tertiary care centres. The need for non-invasive ventilatory support in ALS patients is increasing.