We report a patient with systemic sclerosis who had the unusual development of a large-cell B-cell lymphoma of the base of his tongue. Patients with systemic sclerosis display an increased incidence of malignant neoplasms, particularly, breast and lung carcinomas [Arthritis. Rheum. 36 (1993) 460; Ann. Rheum. Dis. 52 (1993) 531]. The description of this case adds further evidence to the possible interrelationship of systemic sclerosis and the development of malignant neoplasms. A careful review of the English medical literature disclosed that B-cell lymphoma of the tongue in a patient with systemic sclerosis is an association which has not been described previously.