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Choroid plexus papillomas are rare benign central nervous system neoplasms arising from choroid plexus epithelium. They are most often located in the lateral ventricle, followed by the fourth and third ventricles and, rarely, in the cerebellopontine angle.We report an uncommon case of a 17-year-old boy who presented with neck pain that had lasted for more than 1 month, with accompanying pain and numbness in his upper extremities. His conditions included slight dizziness, nausea, diplopia, paresthesia, and an unsteady gait. Magnetic resonance imaging (MRI) showed huge cerebellopontine angle tumor that extended to the front medulla oblongata.Choroid plexus papilloma (WHO I) was diagnosed in this patient.The patient was referred for neurosurgical intervention. The very large neoplasm was subtotally resected.The symptoms of the patient were gradually alleviated after surgery and subsequent radiotherapy treatment, but unfortunately, follow-up of 2 years later revealed that the disease was recurrent and the young man passed away.Neck pain is related to many factors. The case provided an awareness of the origin of severe intracranial disease. It is mandatory to take a thorough clinical assessment with a holistic approach.