Chondromyxoid fibroma (CF) is a benign chondroid and myxoid matrix–producing tumor, often arising in proximal or distal parts of long bones in young adult patients. Local recurrence is common. To date, 25 CFs affecting the jaws have been reported, with the mandible being more involved (76%). A 1-year-old male baby was referred presenting a tumor mass in the left anterior maxilla 2 months ago. The tumor mass caused facial asymmetry. Imaginologic analysis showed a well-delimited maxillary tumor, which extended and compressed the ipsilateral nasal cavity. A complete surgical removal of the lesion was performed. By microscopy, variable cellular areas of fibrocollagenous connective tissue gradually blended with cellular chondromyxoid areas were observed. Immunohistochemistry showed positivity only for vimentin and the Ki-67 labeling index was 6%. The patient is currently under strict monitoring, with no recurrence. CF should be included in the differential diagnosis when assessing maxillary tumors in pediatric patients.