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Cervical ganglioneuroma is a relatively rare benign neurogenic tumor for which standardized evidence-based guideline is lacking. It is more common in women, adolescents, and children and presents as an asymptomatic neck mass. Preoperative imaging and needle biopsy are insufficient to confirm the diagnosis, making postoperative histopathological examination of a specimen the gold standard. Herein, we present the case of a middle-aged man with a giant ganglioneuroma of the neck, discussing the relevant diagnostic and therapeutic characteristics. The patient was a 43-year-old man who had been diagnosed with a left neck mass 6 years prior, without obvious symptoms, received no treatment. One year prior, the patient presented with aggravated speech with content sounds and was admitted at our hospital for relevant examinations and surgical treatment. The patient underwent transcervical tumor resection under general anesthesia. Symptoms such as Horner syndrome, left deviation of tongue protrusion, and dysphagia occurred after surgery. At follow-up 2 months after surgery, symptoms such as left deviation of tongue extension and dysphagia disappeared, and symptoms such as ptosis were alleviated to a certain extent. Surgical resection remains the primary treatment for cervical ganglioneuromas. For large tumors, the transcervical approach can not only completely resect the tumor but also contribute to postoperative recovery.