Spontaneous cerebrospinal fluid (CSF) leak is a condition that commonly presents with unilateral watery drainage from the nose or ear, tinnitus, and stuffy ears or hearing loss. Spontaneous CSF rhinorrhea and otorrhea together are rare. A 64-year-old woman presented at our department with complaints of clear watery rhinorrhea and hearing loss on the right side persisting for 10 months. Imaging and surgery were used to diagnose the condition. Through surgical treatment, she was eventually cured. Review of the literature has shown that patients with both nasal and aural CSF leaks are rare. When a patient presents with both unilateral watery drainage from both the nose and ear, a diagnosis of CSF rhinorrhea and otorrhea should be considered. This case report will benefit clinicians by providing more information to assist with diagnosing the disease.