Dermoid tumors are unusual pouch-like benign growth that may be apparent at birth. Described in this report, dermoid cysts that occurred in the middle ear and mastoid bone which is quite rare. A 3 years old male presented with severe to profound sensorineural hearing loss bilaterally since birth. During cochlear prosthetic device implantation surgery, an incidental intraoperative cyst was found in the postauricular area around 5 cm behind the bony external auditory canal. In this paper, we demonstrate our management for mastoid dermoid cyst with respect to the clinical, radiological, histopathological, and surgical aspects.