Systemic sclerosis (SSc) is a clinically heterogeneous generalized disorder which affects the connective tissue of the skin and internal organs such as gastrointestinal tract, lungs, heart and kidneys. It is characterized by alterations of the microvasculature, disturbances of the immune system and by massive deposition of collagen. Our aim was to to estimate the incidence, prevalence and disease characteristics of SSc in Split-Dalmatia County which has a population of 463,676 Caucasian people. A census of SSc cases for the period 2007-2009 was conducted using hospital records for case identification. Diagnoses were verified by medical record review. All patients were evaluated by the validated American College of Rheumatology criteria for SSc. The exact 95% confidence interval (CI) based on binomial distribution was created for the incidence and prevalence estimates.