| Summary: | Temporal bone involvement in Langerhans Cell Histiocytosis (LCH) is the second most common site of involvement in the head and neck area, with the mastoid and squamous portion of the bone as the most frequent site where LCH manifests. Since there are not many cases reported in the literature, it is possible to state that primary manifestation of histiocytosis affecting the inner ear structures is uncommon. This article reports the case of a patient with involvement of the petrous apex of the temporal bone that was referred to the Fundación Hospital de la Misericordia, and carries out a literature review in order to discuss the manifestation, treatment and outcome of this disease.
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