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Langerhans cell histiocytosis (LCH) is a rare, life-threatening condition that predominantly affects children. It is a diagnostically challenging condition due to several different clinical presentations. Presentations of LCH with otological involvement are considered rare. We report a case where LCH presented a six-month-old boy to our tertiary teaching hospital. The patient presented with a unilateral left postauricular soft tissue mass, with an associated left external ear canal lesion and middle ear effusion, which initially had the working diagnosis of mastoiditis. However, further imaging and molecular testing concluded the final diagnosis as LCH with multisystem involvement. When LCH does have otological involvement, it commonly involves the external and the middle ear, which can often be confused with otitis media and otitis externa. This case report highlights that misdiagnosis of LCH can occur due to its broad presentation. The case report also raises the importance that in cases of unilateral postauricular soft tissue masses with destructive findings on cross-sectional imaging and systemic symptoms and signs, LCH must be a differential diagnosis, and biopsy should be considered.