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A primary thyroid lymphoma is rare among all types of thyroid malignancy. Usually, a thyroid lymphoma is associated with underlying chronic autoimmune thyroiditis. Recently, we experienced a primary thyroid mucosa-associated lymphoid tissue(MALT) lymphoma, with an incidental micropapillary thyroid carcinoma, but lacking evidence of autoimmune thyroiditis. A female patient visited our hospital for further evaluation of a rapidly enlarging, painless thyroid mass which had been stable for 8 years. Lymphocytic thyroiditis or a lymphoma was suspected from fine needle aspiration performed at another hospital. The thyroid function test and other routine laboratory tests were normal. The histopathological findings after a total thyroidectomy revealed a MALT lymphoma with a micropapillary thyroid carcinoma. There was no evidence of chronic autoimmune thyroiditis. This is, to the best of our knowledge, the first case report of a MALT lymphoma arising from the thyroid gland without evidence of chronic autoimmune thyroiditis in Korea