A 74-year-old woman with primary Sjögren's syndrome confirmed by salivary gland biopsy presented with parkinsonism. Magnetic resonance imaging (MRI) of the brain revealed multiple small high intensity lesions in the deep white matter, basal ganglia and pons on T2-weighted images. Treatment with L-dopa failed to improve the parkinsonian features. After the initiation of prednisolone 30 mg/day, the parkinsonian signs and symptoms significantly improved. Some lesions on MRI were decreased in size after corticosteroid therapy. These findings suggest that parkinsonism associated with primary Sjögren's syndrome is at least in part attributable to small vessel vasculopathy such as focal inflammation or edema.