Purpose Study the case of epidermoid cysts in rare parts of the brain and review related literature. Method A case of epidermoid cyst located in the anterior longitudinal fissure cistern of the brain was collected to study the clinical symptoms and signs, imaging, surgical strategy etc. Review relevant literature, summarize the clinical features, diagnosis and treatment strategies of such rare cases. Result This patient with an epidermoid cyst located in the anterior longitudinal fissure cistern of the brain passed the preoperative evaluation. Through the anterior longitudinal fissure approach, the contents of the cyst were completely removed, and the cyst wall was subtotal removed without new complications. Conclusion Epidermoid cysts located in rare intracranial sites had the problem of difficult preoperative diagnosis. The surgical strategy adopted different approaches according to different sites. The occurrence of postoperative complications was not significantly different from that of intracranial epidermoid cysts in common sites.