A 41-year-old female patient was referred to the head and neck surgery department because of a large midline submandibular mass that was diagnostic for functioning thyroid tissue in a totally ectopic location.

Papillary thyroid carcinoma of the pyramidal lobe is rare. We describe a case of a 77-year-old woman who corroborates current literature findings, namely greater presence of adverse prognostic factors. Upper Neck Papillary Thyroid Cancer is a new entity, proposed to group these tumors with potential clinical and therapeutic implications.