The aim of the study was to define main symptoms of clinical appearance and immunoserological profile of male patients with primary Sjögren's syndrome (pSS). Four hundred and ninety-two patients fulfilling the European-American Consensus Criteria for pSS were involved in this study. The mean age of the patients was 55.93 years (55.67 years in women and 56.18 years in men). The female-male ratio was 7:1 (432 and 60 patients, respectively). At the time of the diagnosis of pSS, glandular, extraglandular manifestations (EGMs), and immunoserological parameters were assessed. The major EGMs differ between genders. Arthritis was frequently presented as EGM in both genders, but the ratio was higher in men (68% vs. 42%). Various vasculitis symptoms and lymphadenopathy were more frequent in men than in women, in contrast to Raynaud's phenomenon or autoimmune thyroiditis. Anti-SS-A and anti-SS-B were the most frequent autoantibodies in both genders, although autoantibodies against anti-nuclear factor and extractable nuclear antigens also presented in some patients. In a few cases, there were other specific autoantibodies correlated with EGMs, such as double-stranded DNA, anti-neutrophilic-cytoplasmic antibody, cyclic-citrullinated peptide, anti-thyreoglobuline antibodies, and anti-thyreoid-peroxidase antibodies. Based upon our large cohort of patients with pSS, we conclude that, although the disease is more frequent in women usually about climax, it develops also in men with the predominant symptoms of vasculitis or arthritis besides keratoconjunctivitis sicca or xerostomy.