AIM: To show a case of a giant epidermoid cyst of the posterior fossa treated with a combined microscopic and endoscopic transcranial approach. MATERIAL OF STUDY: A 13-years-old girl presented recurrent episodes of convulsions and vision impairment for years. CT scan and MRI revealed a suprasellar cystic lesion. The patient was operated with a combined microscopic and endoscopic transcranial approach. RESULTS: Postoperative MRI demonstrated complete resection of the mass and pathology confirmed the preoperative suspicion of epidermoid cyst. Post procedure, the patient recovered well and was discharged. At the 4 week follow up her symptoms had resolved completely. DISCUSSION: Epidermoid tumors are congenital lesions with a benign nature. They are the most frequent congenital intracranial lesions. Surgical treatment of epidermoid and dermoid cysts may be challenging. In the recent decades endoscopic endonasal approach (EEA) has been commonly used for the treatment of cranial base lesions because it has several advantages compared to open transcranial approach. We decided to do a transcranial approach combining the use of the microscope with the endoscope because EEA was not possible due to the location of the tumor. CONCLUSIONS: Despite the development of innovative techniques such as the EEA, the transcranial approach remains a viable option, especially when more techniques are combined. In fact, the combination of microscope and endoscope grants the advantages of both tools being very useful to assure a complete excision of intracranial tumors.