Thyroid gland is among the many organs that could be infiltrated in systemic amyloidosis. However, diffuse infiltration of the thyroid gland secondary to systemic amyloidosis associated with Familial Mediterranean fever (FMF) is rare. Here, we present a 49-year-old woman diagnosed with FMF and systemic amyloidosis, who had a large goiter and multiple nodules that developed slowly through the years and was complicated by tracheal compression symptoms and a mild thyroid dysfunction. Multiple fine needle aspiration biopsies of the nodules and the thyroid parenchyma revealed amyloid deposits. We would like to point out that amyloidosis may have a significant impact on the thyroid gland and fine needle aspiration biopsy is a valuable tool for diagnosis.