Sjögren's syndrome (SS) is a chronic autoimmune disease that is characterized by lymphocytic infiltration of the exocrine glands, mainly the salivary and lachrymal glands, usually manifesting with xerostomia and xerophthalmia. Around 50% of patients with primary SS develop systemic complications, lymphoma being the most feared of these. The majority of these neoplasias originate from B cells and are of the non-Hodgkin type. We describe here a rare case of SS in which the patient developed a Hodgkin lymphoma. We also review the literature on this subject.