OBJECTIVE: To find out about the disease characteristics of primary thyroid lymphoma in Pakistani patients. METHODS: In this retrospective study of 20 years duration, we reviewed five diagnosed and/or treated cases of primary thyroid lymphoma at the Aga Khan University Hospital (AKUH), Karachi, Pakistan. Each patient's medical records were reviewed and studied. RESULTS: Mean age of the participants was 62.8±10.2 years with 3 males and 2 females. Two patients presented at the age of 69 and 70 years, and two patients presented at ages of 47 and 58 years. All our patients presented with a rapidly progressing neck mass and dysphagia. Other common presenting complaints in our study subjects included dyspnea, hoarseness of voice, stridor and feeling of globus in the throat. Average duration of presentation of our cases was 3 months, with 2 patient's presenting after a month of development of symptoms, while the remaining two participants presented at four and six months after onset of signs and symptoms. All patient's histopathology was consistent with Diffuse B cell Lymphoma subtype. Out of the five patients, one patient died as a result of complications, one patient opted out of treatment at AKUH due to financial constraints and three patients are doing well with no active complaints and followed-up regularly. CONCLUSION: Primary thyroid lymphoma is a rare condition and can present with neck swelling and compressive symptoms. Diagnosis is based on the histological findings. Larger studies are needed to study the prognosis of the patients.