The clinical and pathologic spectrum of lymphoproliferative disorders affecting the thyroid is diverse and must be differentiated from benign thyroiditis and carcinoma. The clinical presentations include an enlarging neck mass, but patients may also present with symptoms of dysphagia, hoarseness and choking, or a cold thyroid nodule. The histopathologic interpretation requires adequate tissue sampling and proper pathologic interpretation. The recent delineation of new pathological entities such as low-grade malignant lymphoma of mucosa-associated lymphoid tissue (MALT) type has aided in the understanding of the clinical course and management of patients with lymphoma. Advances have been made in the clinical management and treatment of these disorders. Surgical resection of the thyroid mass is not routinely part of the management strategy. The management of low-grade lymphoproliferative disorders of MALT type may include radiation therapy, oral chlorambucil, or intravenous chemotherapy (cyclophosphamide, vincristine, and prednisone). The management of diffuse large B-cell lymphoma is combined-modality therapy with radiation and cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy.