Intraosseous epidermoid cyst (IEC) is a rare, non-neoplastic benign lesion with the most common site involving the distal phalanx. Intraosseous epidermoid cyst is prone to be confused with endophytic chondroma in imaging. In this paper, we reported a case of epidermoid cyst of lunate misdiagnosed as endophytic chondroma in the orthopedic department of our hospital. We used lesion curettage, anhydrous alcohol inactivation and collagen sponge filling to treat the patient. In addition, a thorough literature review was done to summa- rize the clinical features, diagnostic criteria and treatment principles of IEC to provide reference for diagnosis and treatment of epidermoid cyst of lunate.