Abstract
Synchronous presentation of cholesteatoma and tympanomastoid paraganglioma is extremely rare. Due to the overlapping clinical features, the clinical diagnosis of coexistence is difficult. Only two cases of tympanomastoid paraganglioma coexisting with middle ear cholesteatoma have been reported in the literature, but simultaneous presentation of primary external auditory canal cholesteatoma and tympanomastoid paraganglioma has not been reported till date. Coexistence of external auditory canal cholesteatoma and paraganglioma is an incidental diagnosis in this current case. The advancement of imaging techniques could help the diagnosis of this very rare clinical coexistence in preoperative assessment.