Tumefactive demyelinating lesions (TDLs), are a rare demyelinating pathological disease in the central neurological system, which have been proven to be a diagnostic dilemma to neurosurgeons. The clinical presentation and radiographic appearance of these lesions often results in their misdiagnosis as intracranial tumors, such as gliomas, which leads to unnecessary surgical resection and adjunct radiation. In the present study, the clinical and radiographic features of 14 patients with cerebral TDLs who underwent surgical treatment between January 2004 and January 2009 were reviewed and analyzed. The surgical methods used included biopsy and resection, while steroid therapy was indicated when TDLs were confirmed by histopathological analysis. The patients were followed-up and the outcomes were evaluated using the Karnofsky performance scale (KPS). The main clinical presentations included: Hemiplegia (8 cases), increased intracranial pressure (4 cases) and seizures (general in 1 case; partial in 3 cases). On magnetic resonance imaging scans, 12/14 TDL cases demonstrated an isolated local subcortical mass and 6/14 cases (42.9%) demonstrated enhancing veins coursing undistorted through the lesion. The postoperative complications included: Hemiplegia (2 cases) and mortality (1 case). A total of 9 cases underwent microsurgical total resection, and 5 cases received stereotactic biopsy that was followed with high-dose methylprednisolone therapy. The follow-up study demonstrated that 2 cases presented recurrence with multiple sclerosis and the KPS scores for 13/14 patients (92.9%) were = 80. In conclusion, the clinical and radiographic features of TDLs may help to establish the correct diagnosis prior to surgery, in order to avoid unnecessary resection or adjunctive therapy. Using steroid therapy, the majority of patients with TDLs appeared to achieve satisfactory prognosis.