Amyloid goiter is a rare pathology due to massive amyloid infiltration of thyroid tissue, which cause diffuse or localized enlargement of the gland. It can be totally asymptomatic or cause only non-specific symptoms (compression of adjacent structures, tracheal deviation). Thyroid disfunction (hypothyroidism or hyperthyroidism) is rare. It is important to differentiate primitive amyloid goiter from other kinds of amyloid infiltration of the thyroid, such as in systemic amyloidosis. The correct diagnosis can be difficult because of the common presence of amyloid in thyroid neoplasms, such as medullary thyroid carcinoma (MTC). The Authors present a case of primitive amyloid goiter, that needed immunohistochemical analysis for a correct diagnosis, and a review of the concerning literature.