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Primary thyroid lymphoma (PTL) is a rare malignancy of the thyroid gland. It usually presents itself as a very rapidly growing mass, and respiratory distress is commonly the first finding. Ultrasound guided fine needle aspiration biopsy and surgical biopsy can help with its diagnosis. The treatment is radiotherapy and chemotherapy. Surgical treatment is only indicated in cases which tracheal compression is severe. In this report, we present a primary thyroid lymphoma case, which presented with progressive respiratory distress, and dramatically went into remission with medical therapy, along with a literature review.