We report the case of a 48-year-old woman who presented with bilateral parotid gland enlargement as the initial manifestation of primary Sjögren syndrome. Magnetic resonance imaging demonstrated multiple areas of low-intensity signal mixed with foci of high-intensity signal, a finding suggestive of Sjögren syndrome. Autoimmune blood tests for Sjögren syndrome were positive, and a sublabial biopsy confirmed the diagnosis. Investigations for other conditions, including human immunodeficiency virus infection, were negative. After 5 years of follow-up, the parotid swellings remained essentially unchanged. We discuss the etiology of multicystic parotid gland pathology, its involvement in Sjögren syndrome, and the risk of lymphoma.