<![CDATA[Langerhans Cell Histiocytosis (LCH) is one of the rare disease with atypical clinical manifestations. LCH must be watched out for and considered a differential diagnosis of the other otolaryngology diseases. A 2-years-10-months old boy with a crusted lump in the right ear canal accompanied by a lump behind the right ear that is expanding, bleeding for 3 months, and the patient experience loss of weight. Examination of the ear found a black mass covered with crusts with a firm, fixed consistency, filling the lateral one-third of the right external acoustic canal to the right auricle concha. A solid mass was obtained from the lateral one-third of the right external acoustic canal on a head CT scan, medially obliterating the external acoustic canal to the right internal acoustic canal. FNAB found nonspecific inflammation. Performed mass excision surgery and anatomical pathology examination. The patient was referred to the pediatric haemato-oncology division and underwent chemotherapy. Langerhans cell Histiocytosis in the temporal bone is a rare disease with atypical clinical manifestations, so it should be considered as one of the differential diagnoses if a mass is found in the external acoustic canal.]]>
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