Hypoglossal schwannoma in the submandibular region

Hypoglossal nerve schwannomas originating extracranially and mimicking a submandibular salivary gland tumour are extremely rare. A 55-year-old woman presented with a painless, gradually increasing swelling in the right submandibular region for the past 1 year. Fine-needle aspiration cytology and contrast-enhanced CT of the swelling showed features of submandibular gland malignant lesion. Intraoperatively, the right submandibular gland with a hypoglossal nerve swelling was noticed. Right submandibular gland along with the hypoglossal swelling were excised with adequate margins. However, the postoperative histopathology was reported as hypoglossal nerve schwannoma and a normal salivary gland. Accurate preoperative diagnosis of hypoglossal schwannomas may be challenging. A high level of suspicion must be sought for in cases with unusual clinical presentations and imaging characteristics. Herein, we report a rare presentation of submandibular hypoglossal schwannoma along with its clinical features and its management.