| Summary: | The uncommon occurrence of primary squamous carcinoma of the submandibular gland makes it a challenge to diagnose and treat. Clinical as well as histopathological assessments are key elements for the diagnosis. Although no clear treatment guidelines exist, surgical excision with a neck dissection is the cornerstone of the treatment with or without adjuvant therapy. In this paper, we report a rare case of a primary squamous carcinoma in an 82-year-old lady with no history of smoking or alcohol use, who presented with a right sided cervical swelling of 3-month duration. An ultrasound guided fine needle aspiration cytology was negative, as well as a panendoscopy with systemic biopsy of the base of tongue and the homolateral palatine tonsil. In addition, a blind fine needle aspiration cytology from the mass during the panendoscopy was performed and was positive for squamous cell carcinoma. A PET scan showed hypermetabolism of the right submandibular gland with no distant lesions. Therefore, a submandibular gland excision was performed with a frozen section histopathological examination in favor of squamous cell carcinoma, therefore the intervention was completed by a selective neck dissection. High clinical suspicion should be present while dealing with this rare entity, as well as not underestimating the poor outcomes associated with it.
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