We report a rare case of an olfactory ensheathing cell (OEC) tumor with literature review. A 48-year-old man presented after experiencing two episodes of generalized seizures. Brain imaging showed a well-enhanced solid mass with a cystic component in the anterior cranial fossa. However, the patient did not report anosmia. The mass was totally removed through bi-frontal craniotomy. Histopathological examination showed that the mass consisted of whorling spindle cells with Antoni type A and Antoni type B cellular patterns. The immunohistochemical stain was strongly positive for S-100 protein and negative for epithelial membrane antigen and Leu-7. The final diagnosis was OEC tumor.