Chondrosarcomas are infrequently found in the head and neck region. In a review of nonepithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx, only 4% were chondrosarcomas. The peak incidence occurs in the fourth decade of life. Only four cases of head or neck chondrosarcoma in children less than or equal to 10 years of age have been described in the literature, and only two of these were primary nasal cavity tumors. We present in this report a case of a 5-year-old boy treated with adjuvant chemotherapy after complete resection of a nasal cavity chondrosarcoma, and a review of the literature on this topic.