Sjögren's syndrome is an autoimmune exocrinopathy of unknown etiology. It is characterized by a chronic inflammatory process that leads to functional impairment and destruction of lachrymal and salivary glands. There is a primary and a secondary form of the disease; the latter accompanies a well defined connective tissue disease. As far as we know, there are no previous reports of primary Sjögren's syndrome in Venezuela. The main purpose of this study was to present the clinical findings and outcome of a population of patients diagnosed and followed in our hospital. A population of fifty-four patients predominantly females (96%) with a mean age of 42 (range 24 to 84) was studied. The presence of articular symptoms was the most common extraglandular manifestation (87%), followed by enlargement of parotideal glands (52%). Parotid enlargement and renal disease were observed with a higher frequency than previously reported in the literature. Recurrent enlargement of parotideal glands has been related to lymphoid malignant transformation in these patients. Our findings seem to suggest that the pattern of clinical expression of primary Sjögren's syndrome may be influenced by the genetic make-up of the population under study, and possibly by local environmental influences. Two of our cases developed pseudolymphoma, a transitional stage between the benign lymphoproliferation of primary Sjögren's syndrome and lymphoma. Furthermore, in this relatively small sample six patients have died during a short follow-up period, suggesting a potentially more aggressive course of the disease in our patients.