Primary thyroid lymphomas (PTL) are very rare tumors, typically presenting as a rapidly enlarging, painless thyroid mass, which may cause pressure symptoms of the aerodigestive tract. Preoperative diagnosis can be established by using modern imaging methods (mainly ultrasonography) and FNA with immunohistochemistry and flow cytometry. Open (surgical) biopsy may be indicated to confirm the diagnosis and identify the subtype of PTL. Therapeutic strategies are distinct from other thyroid malignancies, and include local therapy alone (surgery or radiotherapy or surgery plus radiotherapy) or - most commonly - combined multimodality treatment (mainly chemoradiation therapy). Management should be tailored to the individual patient; treatment-related morbidity should be taken into account. A high index of suspicion from the part of the clinician is required to achieve early diagnosis and prompt treatment of this potentially curable thyroid malignancy.