Superior semicircular canal dehiscence syndrome (SCDS) is a rare disease caused by dehiscence in the bone overlying superior semicircular canal. Patients with SCDS present vestibular and auditory manifestations including dizziness and hyperacusis. These manifestations can be controlled by surgical repair of the dehiscence such as plugging or resurfacing the defect. We report a case of a 25 year-old man with SCDS who underwent surgical repair of dehiscence through middle fossa approach.