Epidermoid tumors, sometimes called pearly tumor, because of the appearance of their outer surface, represent about 0.2%-1.8% of all the primary intracranial tumors1 and are benign developmental lesions. However in 1912, st et al2 found a case of primary intracranial squamous cell carcinoma arising in an epidermoid cyst in autopsy and reported it. Hereafter, case reports about primary intracranial squamous cell carcinoma appeared in succession, especially after computed tomography (CT) and magnetic resonance imaging (MRI) were widely applied in clinical medicine. To our knowledge, few cases have been reported in our country until now. We present a case of intracranial squamous cell carcinoma developing in remnant of an epidermoid cyst 11 years after the operation resection with a review of the literature.