Nasal chondromesenchymal hamartoma (NCMH) is a rare nasal benign tumor, which arises from the nasal cavity or paranasal sinuses. In this article, we present a five-year-old male patient with rhabdomyosarcoma unity in remission that emerged with nasal obstruction. Synchronous diagnosis of pediatric tumors such as pleuropulmonary blastoma in the literature is a remarkable finding. We found a mass within the left nasal cavity originating from superior portion of nasal septum, extending to the olfactory cleft and resected all tumor via endoscopic surgical approach. Histopathological diagnosis revealed that NCMH contained cartilaginous and mesenchymal components. In conclusion, NCMH is a rare surgically treated benign tumor that can be synchronously diagnosed with pleuropulmonary blastoma and should be kept in mind for differential diagnosis of unilateral pediatric nasal mass.