A patient with swelling of the left parotid gland of four-months' duration, sicca syndrome (xerophthalmia and xerostomia) and a history of progressive systemic sclerosis with an incomplete form of the CREST syndrome was referred to our department. On ultrasound a parotid mass of reduced echogenicity without any enlarged cervical lymph nodes was found. Ultrasonographically guided fine-needle biopsy could not provide any definitive diagnosis. After partial parotidectomy with complete tumor removal the histologic exam showed an extramedullary plasmacytoma with concurrent non-necrotizing granulomatous sialadenitis of the parotid gland. Complete systemic work-up excluded multiple myeloma, leukemia, lymphoma and sarcoidosis. Post-operative radiotherapy of the left parotid region and left neck including the supraclavicular lymph node area was performed. Six months after surgery an aggressive B-cell non-Hodgkin's lymphoma was diagnosed.