Background. Sjögren's syndrome (SS) represents autoimmune disease characterized by chronic inflammation, destruction and insufficiency of exocrine glands, particularly salivary and lacrimal glands, accompanied by dryness of mouths and eyes. Diagnostic work-up involves clinical laboratory tests, radiography, scintigraphy and bioptic histopathological examination. Examination of small salivary glands in the biopsy of the lower lip represents a 'golden standard' of diagnosis of SS, concerning the fact that the growth and the disfunction of salivary and lacrimal glands occurs in different pathologic states. Methods. Resected specimens of the lower lip were obtained from 47 patients with clinical diagnosis of SS. After standard histopathological treatment, slices were hematoxylin and eosin stained. Immunohistochemistry against smooth muscle actin was performed using LSAB+ method (AHSMA-M7558, DAKO 1:50). On the basis of generally accepted histopathological diagnostic criteria the results were categorized as: findings suspicious for SS; findings compatible with the diagnosis of SS (mild, moderate and high degree of inflammation); nonspecific inflammatory reaction and nonrepresentative biopsy samples. Results. Diagnosis of SS was confirmed in 32% of cases. In 2% of cases findings were suspected for SS, in 36% of cases findings were compatible with the diagnosis of nonspecific inflammation, and in 30% of cases material was not representative. Conclusions. By the biopsy of salivary glands of the lower lip the diagnosis of SS was confirmed in 50-60% of cases. Upon the precise diagnostic criteria it was also possible to determine the intensity of inflammation and tissue destruction in SS and identify other pathological conditions, which justified the biopsy. Surgical technique had to be adequate in order to obtain representative number of small salivary glands. In the presented material 30% of specimens were nonrepresentative which was very high percentage compared with literature data. This was most probably the reason why the diagnosis of SS was confirmed in only 32% of cases, i.e., in every third patient.