The present case report concerns a 64-year-old female who referred with an enlargement mass of right side of neck(submandibular angle) since 4 months ago. Mass resection specimen grossly revealed firm red-brown well-circumscribe mass. Cut sections showed nonhomogeneous tissue with multiple areas of hemorrhage associated with two regional lymph nodes. Microscopic examination mostly exhibited zell ballen pattern with pleomorphic oval to polyhedral tumoral cells which contained eosinophilic granular cytoplasm and hyperchromic nuclei surrounded by sustentacular cells. In subcapsular area of lymph node, a focus of metastatic tumoral cells was also seen. Ultimately, histopathologic diagnosis revealed a very rare malignant paraganglioma of carotid body.