Objective To investigate the clinicopathological features of calcifying pseudoneoplasms of the neuraxis (CAPNON). Methods The data of 3 CAPNON patients diagnosed in The Second Affiliated Hospital of Naval Medical University (Second Military Medical University) from Sep. 2019 to Sep. 2021 were collected, and their clinical imaging characteristics, histological morphology and immunohistochemical and molecular phenotypes were analyzed. Results The 3 CAPNON patients, 1 male and 2 females, were aged 15, 33 and 56 years old. Their clinical manifestations included limb muscle atrophy, intermittent low back pain and intermittent headache, and the imaging findings showed space occupying lesions. All 3 cases were treated by surgical resection. Histological observation showed nodular cartilage-like stroma and spindle-shaped stromal cells, with calcification, ossification and gravel bodies, histocyte reactions around the lesions and foreign body granuloma formation. Immunohistochemistry showed CD68 (+) in histocytes, vimentin and epithelial membrane antigen (+) in spindle mesenchymal cells. Genetic testing showed c.1924G>A missense mutation in somatic Fms-related tyrosine kinase 4 gene in 1 patient. No recurrence was found during a follow-up of 17-41 months. Conclusion CAPNON is a rare neuroaxial fibroskeletal lesion. The diagnosis of CAPNON should combine histological morphology and clinical imaging data, and molecular pathological examination may help to understand its pathogenesis.