The most common symptoms of eosinophilic granuloma are local tenderness and an enlarged skull mass. The presence of epidural hematoma is a very rare symptom of eosinophilic granuloma. To our knowledge, this is only the second reported case of eosinophilic granuloma with epidural hematoma. A 2-year-old boy with a soft tumor on the occipital scalp, palpable at the age of 3 months, yet with no obvious history of trauma, was admitted due to a sudden onset of loss of consciousness. A brain computed tomography scan showed a lytic lesion on the occipital skull with a large epidural and subcutaneous hematoma, causing brain compression. He underwent an emergency craniectomy with removal of both the tumor and hematoma. The patient regained consciousness and had no residual neurological damage. Pathological reports showed abnormal proliferation of Langerhans’ cells, eosinophilic cells and multinucleated cells. A whole-body bone nuclide scan revealed no other bone lesions. The patient was discharged uneventfully. The causes of hematomas are not very clear. They may be due to tumor necrosis or minor trauma. In our presented case, the cause of the epidural hematoma may have been tumor bleeding which ruptured into the epidural space. A solitary eosinophilic granuloma of the skull with acute epidural hematoma and loss of consciousness is extremely uncommon. Craniectomy with removal of the tumor and hematoma decompression may produce good results.

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