Cholesteatoma involving the petrous apex is an uncommon lesion that is diagnosed clinically by such noninvasive methods as temporal bone CT scanning, MRI, and angiography. These cholesteatomas originated as a congenital primary lesion or secondary to an acquired lesion. Obviously, the diagnosis and treatment of petrous apex cholesteatoma is a difficult surgical challenge. This paper reviewed the therapeutic details of three cases of cholesteatoma involving the petrous apex.