We present an exceedingly rare case of chondromyxoid fibroma of the nasal cavity and palate. This rare tumor usually occurs in the metaphyses of the long bones. Our patient's tumor was diagnosed by biopsy, and it was resected en bloc. At the 2-year follow-up, the patient was doing well with no signs of recurrence. We review the pathologic characteristics, differential diagnosis, and treatment of chondromyxoid fibroma of the nasal cavity.