AbstractAlthough a thyroglossal duct cyst (TDC) is a common cyst occurring in the neck, carcinomas arising in the TDC are a rare event. To date, approximately 100 cases have been reported, the majority of them being papillary carcinomas. Squamous cell carcinomas (SCC) are very rare, and Only nine cases have been reported so far in the literature.We present a 55-year-old female patient with a SCC in a TDC to highlight the clinical and pathological features of this condition.