McCune-Albright syndrome is a disorder of fibrous bone dysplasia complicated by skin pigmentation and endocrine abnormalities. Although temporal bone lesions are rare, surgical treatment is required when external auditory canal (EAC) stenosis develops. However, no consensus has been reached regarding surgical approaches. To safely perform surgery for temporal bone malformations, knowing the exact location of the critical organs in the temporal bone in relation to the bony lesion is important. Otherwise, intraoperative orientation may be lost owing to differences from the normal anatomy. Although image-guided surgery systems (IGSSs) would be useful in surgery for temporal bone malformations, few studies have reported on the use of IGSS in temporal bone surgery. In this report, we describe a case of McCune-Albright syndrome with EAC stenosis that was safely treated using IGSS-assisted temporal bone surgery.