Primary Sjogren's syndrome (pSS) is a chronic autoimmune disorder of the exocrine glands. The diagnosis is largely based on keratoconjunctivitis sicca and xerostomia in the presence of anti-SS-A and/or SS-B antibodies. Anticentromere antibodies (ACA) have occasionally been reported in patients with pSS. We describe two patients with pSS associated with ACA, initially diagnosed as limited systemic sclerosis. Symptoms at the time of initial presentation were dry eyes and mouth, arthralgias, and Raynaud's phenomenon. Both patients developed small vessel cutaneous vasculitis, parotid enlargement, low C4 complement levels, positive rheumatoid factor, and lymphoma. These findings suggest that patients with pSS who have ACA may be a subgroup of patients at increased risk of extraglandular systemic manifestations and lymphoma.