Chondromyxoid fibroma is a rare benign tumor that usually occurs in the long bones. A 2-month newborn presented with huge masses in the both nasal cavities, which turned out to be chondromyxoid fibroma. The masses originated from both inferior turbinates. Total turbinectomy on the left side and submucosal mass excision on the right side were performed. No recurrence or new lesion was observed during the 2 years of follow up. We report here on a rare case of nasal cavity chondromyxoid fibroma in a neonate and we review the relevant literature.