Langerhans cell histiocytosis (LCH) is a rare disease that may affect multiple organs. The etiology of LCH remains unclear to date. It is currently believed that clonal accumulation and proliferation of CD1a-positive Langerhans cells are causative. The term LCH or histiocytosis X refers to three separate illnesses (listed in order of increasing severity): eosinophilic granuloma, Hand-Schüller-Christian disease and Letterer-Siwe disease. A seven-month-old boy presented with history of recurrent bilateral otitis media and rash and seborrheic areas on his scalp. Two days prior, his mother noticed a small lump over the right mastoid. Lateral skull X-ray (Schüller) was evidence for lytic lesion on right temporal bone. The computerized tomography scan showed inflammatory changes with bone erosion. During surgical exploration, fragile slightly yellowish tissue with necrotic areas was found that was determined as LCH on histology. Chemotherapy was subsequently initiated. The initial presentation of LCH with bilateral ear and skull involvement is a very rare condition. The signs and symptoms of otologic histiocytosis can mimic those of acute and chronic infectious ear disease. Only a surgically obtained biopsy leads to definitive diagnosis and appropriate therapy.