The Rationale: Although 25%–45% of schwannomas are originating from the neck, carotid space schwannomas are extremely rare. Patient Concerns: We report a rare case of huge-sized schwannoma in a 20-year-old student who presented with a symptomless large carotid space mass. Diagnosis: Cervical magnetic resonance imaging (MRI) with contrast revealed a huge, well-defined mass measuring 13.7 cm × 6.4 cm × 4.1 cm. Cervical MRI along with brain MRI were consistent with neurofibromatosis type II. Treatment and Outcomes: Preoperative tracheostomy and wide local excision of schwannoma via a transcervical approach were performed with nerve preservation. Takeaway Lessons: The scarceness of cervical sympathetic chain schwannoma made this case of our patient very interesting to report. Moreover, our patient's huge tumour size is extremely rare, and we could not find any similar cases in the literature.