To investigate the clinical experience of glomus jugulare paraganglioma by presenting a case of giant glomus jugulare paraganglioma. The clinical data of 1 case of giant glomus jugulare paraganglioma with unilateral anacousia and pulsatile tinnitus admitted to our department was retrospectively analyzed, and the relevant literature was reviewed to summarize the characteristics of the disease. The tumor tissue in the jugular venous foramen region was completely resected, with complete preservation of the facial nerve during the operation. There was no tumor recurrence during the 2-year postoperative follow-up period. With nonspecific clinical symptoms and a high rate of early misdiagnosis The giant glomus jugulare paraganglioma case only manifested as symptoms of unilateral anacousia and pulsatile tinnitus is clinically rare. The intraoperative safe resection of the tumor, maximum preservation of facial nerve function remains the focus of surgery.