Objective To study pathological features, diagnosis and differential diagnosis, and treatment of sclerosing epithelioid fibrosarcoma ( SEF ). Methods Clinical manifestations, pathological morphology, immunohistochemical staining, and treatment of cervicothoracic vertebrae SEF in 1 patient were analyzed with related literature review. Results The case ( male, 62-year-old ) of cervicothoracic vertebrae SEF was first reported. Primary tumors located in cervical vertebrae and thoracic vertebrae, inducing spinal cord compression. A large amount of irregular cord-like trabecular collagen was found in tumors. Rarefaction of tumor cells and spindle cells around the collagen was observed, among which were epithelial cells. Calcification and ossification were found in certain areas of the tumor. Immunohistochemical staining showed that the tumors were positive in Vimentin ( 3+ ), CD99 ( 3+ ), Bcl-2 ( 2+ ), KI-67-LI ( 15%+ ), while negative in SMA, Desmin, NF, CgA, S-100, HMB-45, CD34, EMA, CK8, CK19, CK7, p53, CK, CK5, GFAP, CD68, CD21, CD163, CD1a and S-100. Conclusions SEF is an extremely rare type of fibrosarcoma. It can be confirmed by pathological examination and immunohistochemical study. Surgical treatment is the main option.