Sjögren's syndrome is a relatively uncommon condition in the paediatric age group. The youngest child reported thus far was a 5-year-old girl. This article reports the case of a 2-year-old girl admitted for recurrent infections of the respiratory tract with diffuse pulmonary interstitial infiltrations and a progressive swelling of the parotid glands. The clinical features and the results of laboratory investigations, including parotid and hepatic biopsies, chest X-rays and sialography helped to establish the diagnosis of Sjögren's syndrome. Clinical, immunological and genetic characteristics of Sjögren's syndrome are reviewed.