A 63-year-old woman was referred to a department of oral and maxillofacial surgery because of slowly increasing enlargement of the tongue. Histopathologic examination of a biopsy showed the presence of amyloid. Further physical and laboratory studies disclosed the presence of multiple myeloma (Kahler's disease). A few years before, the patient had been operated for metacarpol syndrome of both hands, most likely because of amyloid deposition. The patient underwent a stam cell transplantation. After one year follow-up the patient is still alive. There was no distinct regression of the macroglossia.