The coexistence of paraganglioma with cholesteatoma is a very rare clinical disorder. The clinical presentations are nonspecific. There may be radiological characteristics of either cholesteatoma or paraganglioma in the middle ear area, but the diagnosis of the coexistence of the 2 is usually made only postoperatively. Here is such a case that is made more interesting not only because it initially presented with conductive hearing loss but also because the clinical picture mimicked chronic otitis media. The patient underwent postauricular tympanomastoidectomy with extended facial recess approach to remove the tumor. No evidence of recurrence and complications were noted.