Intraosseous epidermoid cyst (IEC) is a rare, non-neoplastic benign lesion. The most common site is the distal phalanx. Intraosseous epidermoid cyst is easily confused with endophytic chondroma in imaging. In this paper, we reported the diagnosis and treatment of a case of Lunate intraosseous epidermoid cyst misdiagnosed as Lunate endochondroma in the orthopedic department of our hospital, in order to provide reference for the research and diagnosis and treatment of Lunate intraosseous epidermoid cyst in the later stage. So far, it is also the first case of epidermoid cyst in Lunate bone. A 62-year-old male was admitted to hospital because of pain in the left wrist and limitation of movement for 2 months. X-ray, CT and MRI examination of the left wrist showed bone destruction of the Lunate bone of the left wrist, nodular high-density shadow in the center, increased bone density at the edge, and uneven long T1 and T2 signal shadow of the Lunate bone of the left wrist. The operation was performed with one-stage dorsal approach for left Lunate osteotomy and exploration, focus curettage, anhydrous alcohol inactivation and collagen sponge filling. The operation was smooth, the incision healed in one stage, and was fixed with plaster support for one month after operation. One month after operation, there were no signs of local recurrence and pain symptoms were significantly relieved.Three months after operation, no perioperative and postoperative complications occurred, pain symptoms disappeared completely, wrist joint activity was normal.