Cervical sympathetic chain schwannoma (CSCS) is an extremely rare benign tumor, and it is a diagnostic challenge. We report a case of 45-year-old female who presented with a solitary right cervical swelling with clinical features of Horner's syndrome (HS). She was evaluated with computed tomography, magnetic resonance imaging, and angiography. Surgical excision of the lesion was performed, and the histological examination revealed the diagnosis of schwannoma. Herein, we review the presentation, imaging characteristics, and operative considerations of a patient with a large CSCS, presenting with HS.