Nasal chondromesenchymal hamartoma (NCMH) is an extremely rare benign pediatric tumor that was described in 1998. Only 19 cases are reported in the literature. We present a 15-year-old girl with nasal obstruction and recurrent sinusitis. Her medical history was significant for a rare ovarian tumor and pleuropulmonary blastoma. CT demonstrated a partially calcified soft-tissue mass obstructing the nasal cavity. The patient underwent endoscopic surgical excision. Histologic and immunohistochemical analyses of the tumor were consistent with NCMH. The imaging characteristics of the tumor are reviewed. NCMH may be part of a syndrome associated with other pediatric neoplastic and dysplastic disease.