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There are several congenital anomalies of the mastoid and external ear. The external ear andmastoid form during the developmental process through the differentiation of ectoderm andmesoderm, and anomalies can arise at various stages. While most congenital anomalies can beeasily diagnosed through physical examination, some cases, such as congenital mastoid choles-teatoma, may delay diagnoses. We present a successfully treated case that involved the removalof three simultaneous congenital anomalies of ectodermal origin, which included preauricularfistula, congenital mastoid cholesteatoma, and postauricular epidermal cyst, all found on oneear in a single patient.