A case of amyloid tumor of the palate is reported in a 10-year-old girl. The past history, the detailed general clinical and laboratory examinations, and biopsy specimens collected from the rectal mucosa, gingiva, tongue, and skin excluded other forms of localizations of amyloidosis. The isolated amyloid tumor is an exceptional form of amyloidosis, and in the available literature we have not found mention of its localization on the palate.