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We present imaging and clinical findings of a case with a duplicated external auditory canal, which led to misdiagnosis secondary to lipomatous content on imaging studies prior to surgery. It is a very rare congenital anomaly. This entity is usually misdiagnosed not only clinically but also radiologically. This leads to a delay and failure in diagnosis and treatment. To the best of our knowledge, herein is the first case report of the duplication of the external auditory canal presenting with dominant lipomatous imaging features. In this report, we aim to draw attention to the atypical presentation.