Chondromyxoid fibromas are uncommon tumors most often seen in long bones of adolescent and young males. Involvement of craniofacial bones is extremely unusual, with sporadic case reports described in the literature. We describe the first case of chondromyxoid fibroma arising in the nasal septum with local destruction and expansile growth into the ethmoid bone and inferior turbinate in a 60-year-old female. The fortuitous discovery of this otherwise asymptomatic lesion and its follow-up are detailed. The literature is reviewed and salient clinical, radiographic, and pathologic correlative findings are emphasized.
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