Objective To report a case of communicating giant epidermoid cyst of nasal orbital skull base and the relevant literatures were reviewed in order to investigate the treatment of communicating epidermoid cyst of nasal orbital skull base and improve the clinicians" understanding of the rare disease. Methods One patient with communicating giant epidermoid cyst of nasal orbital skull base was performed with resection of tumor plus reconstruction of anterior skull base. During surgery, the tumor bottom wall was located in the residual medial posterior orbital wall, and the bone defect of the skull base behind the tumor was observed. After complete resection of the tumor, cerebrospinal fluid leakage was found at the junction of the superior orbital fissure and the anterior skull base, so a pedicled temporal musculocutaneous flap was transferred to fill and reconstruct the anterior skull base. The remaining cavity was transplanted adipose tissue to restore the normal shape of the nasal-orbital-skull base. The tumor was sent to the department of pathology for diagnosis. Results The patient"s nasal and orbital appearance returned to normal, and the compression symptoms disappeared after satisfying surgery. Histological examination suggested for epidermoid cyst. There was no recurrence during 4 years follow-up. Conclusion Surgical resection is the first choice in treatment of the communicating giant epidermoid cyst of nasal orbital skull base. In the case of a cranial base defect, reconstruction and repair of the skull base structure is essential to reduce postoperative complications and improve the quality of life.