Introduction:This study aims to investigate the pathological features of a patient with paraganglioma in the lumbar spinal canal.Case presentation:The patient was 36 years old male with electrical pain in the waist and buttock which occurred intermittently, and was not relieved under oral use of analgesics. Immunohistochemical staining was performed to diagnose the disease. The results revealed that the size of the tumor was 3.4 × 1.6 × 1.4 cm. The hematoxylin and eosin (H&E)-stained tumor cells mainly presented with an organ-like arrangement under low power microscope, showing prominent chrysanthemum-like, pseudo glandular or pseudo papillary arrangements. The surrounding area of the nest presented with sinusoids, and fibrosis and focal calcification could be observed in the interstitial space among the lesions. Immunohistochemistry results showed that the chief cells were positive for neuron-specific enolase (NSE), Syn and CgA, and Sertoli cells were positive for S-100.Conclusion:Paraganglioma is a very rare malignant tumor. This tumor should be distinguished from ependymoma, meningioma and hemangioblastoma, to avoid misdiagnosis, and missed diagnosis.