Malignant external otitis (necrotizing otitis externa) is an infectious process which occurs most often in middle-aged and elderly diabetic patients and is characterized by cultures positive for Pseudomonas and granulation tissue at the junction of the bony and cartilaginous portion of the external auditory canal. Epidermoid carcinoma of the external auditory canal is also seen most often in the middle-aged and elderly population. To the best of our knowledge, no case of acute necrotizing otitis externa occurring concomitantly with epidermoid carcinoma of the external auditory canal has been documented. A case is presented and discussed here and the importance of biopsy of the external auditory canal is stressed. Theoretical considerations of the possible relationship between these two disorders are discussed. This case illustrates the need to discontinue the use of the term "malignant external otitis" and replace it with the term "necrotizing otitis externa."