A 44-year-old woman had a four-month history of enlargement of her thyroid. Evaluation revealed a rare disease: nodular, poorly differentiated lymphocytic lymphoma of the thyroid (NPDL), stage IE. A review of the literature revealed that most thyroid lymphomas are diffuse histiocytic (DH). Treatment of thyroid lymphocytic lymphoma and survival rates have been quite variable between institutions. There are no large studies to guide the physician in making a management plan to treat thyroid NPDL. Our patient was treated with a combination of chemotherapy and radiation therapy (XRT).