Primary thyroid lymphoma (PTL) is a rare malignancy originating in the thyroid gland. It can manifest as a rapidly growing mass within the mediastinum, potentially compressing the trachea and causing respiratory difficulties. Diagnosing PTL can be challenging due to its rarity, impacting the available treatment options. The choice of surgical intervention depends on the location, depth, and extent of the invasion. If the tumor has penetrated the tracheal mucosa, then complete removal is required, but this may result in serious complications. Here, we report a distinctive case of PTL involving the trachea, and further complicated by severe airway obstruction and perforation.