Background: Primary intraosseous schwannoma is a rare benign tumor derived from schwann
cells that cover myelinated nerve fibers, accounting for less than 0.2% of all primary bone tumors. Because of its rarity, the new World Health Organization classification of bone tumors has deleted it.
Case presentation: A 29-year-old woman presented with a history of intermittent pain on her right ankle over three years. Radiological examination revealed a multilocular cystic and lytic lesion in the distal tibia. Histologically, the tumor was composed of spindle shaped cells, similar to soft tissue neurilemmoma. The patient underwent a surgical resection of the intraosseous lesion. After a 38-month follow-up, the symptoms were significantly relieved and there was no clinical or radiological recurrence.
Conclusion: Primary intraosseous schwannoma is a rare benign bone tumor. The pathologists should be aware of the incidence of the tumor, although WHO has deleted it.