OBJECTIVE: To describe an unusual presentation of Langerhans cell histiocytosis in the craniofacial skeleton in a patient previously diagnosed with Pott's disease.

METHODS:
               DESIGN: Case report.
               SETTING: Tertiary care center.
               PATIENT: One (1)

RESULT: A 30-year-old male who previously underwent cervical spine surgery for Pott's disease, presenting with watery ear discharge and mandibular resorption was initially diagnosed with tuberculosis. On MRI, a mildly contrast-enhancing soft tissue mass involving the left infratemporal and middle cranial fossae consistent with residual or recurrent tumor as well as an inflammatory process was seen. He underwent transtemporal excision of the mass with external auditory canal blind sac closure and obliteration. Final histopathology revealed Langerhans cell histiocytosis. A review of slides of the specimen from the previous spine surgery was done and signed out as Langerhans cell histiocytosis.

CONCLUSION: Langerhans cell histiocytosis occurs rarely, but has frequent head and neck manifestations. It may also be confused with other diseases in the head and neck, such as tuberculosis, in this case. Otorhinolaryngologists and head and neck surgeons should be well aware that isolated lesions in the bony framework of the head and neck should include Langerhans cell histiocytosis in the differential diagnosis. Early detection is the key to preventing disease progression and instituting timely definitive management.