Objective To analyze the clinical characteristics of malignant paraganglioma of the parapharyngeal space, and to explore its diagnosis and treatment.Methods Clinical data of 8 patients with pathologically confirmed malignant paraganglioma of the parapharyngeal space hospitalized in Department of Otolaryngology Head and Neck Surgery, the Chinese PLA General Hospital between Jan 2010 and Dec 2021 were retrospectively analyzed. There were 3 males and 5 females, aged from 21 to 57 years old. Without specific clinical symptoms, preoperative enhanced computerized tomography/magnetic resonance imaging and digital angiography in all the patients revealed imaging features of parapharyngeal space hypervascularized tumors with close relations to the large vessels of the neck.Results Cervical or combined craniovertebral approaches were adopted in all patients for tumor resection and regional lymph node dissection. Of them, 5 underwent one operation, 2 had two operations and 1 received three operations. There were no cerebrovascular complications in all patients. Local lymph node metastasis occurred in 7 patients and distant metastasis in one. One patient underwent postoperative radiotherapy and one underwent postoperative chemotherapy. None of the patients died.Conclusions Malignant paraganglioma of the parapharyngeal space is rare and its main treatment is surgery. At the same time, a comprehensive treatment plan needs to be developed according to the characteristics of the tumor and specific situation of the patient.