Objective To analyze the pathological features, clinical manifestations, diagnosis and treatment process as well as misdiagnosis of nasopharyngeal malignant tumors in children and adolescents, and to discuss the problems worth attention in the diagnosis and treatment.Methods A retrospective analysis was conducted on 1 480 cases of nasopharyngeal lesions in children and adolescents diagnosed and treated in the first affiliated hospital of Nanjing medical university (Jiangsu provincial people’s hospital) from January 2015 to December 2022. Among them, 20 patients suffered from malignant tumors, their clinical manifestations, pathological types and diagnosis and treatment were analyzed, the problems and omissions in the diagnosis were discussed.Results The nasopharyngeal malignant tumors included 16 cases of squamous cell carcinoma, 2 cases of lymphoma, 1 case of papillary adenocarcinoma and 1 case of chordoma. Four of 7 cases with initial nasal symptoms were misdiagnosed as adenoid hypertrophy, 2 of 5 cases with initial otologic symptoms were misdiagnosed as simple secretory otitis media, and none of 8 cases with initial neck symptoms was misdiagnosed. The total misdiagnosis rate was 30%(6/30). The disease course of misdiagnosed patients was longer than that of non-misdiagnosed patients, and the difference was statistically significant (Z=-3.160, P<0.01). The duration of the disease before diagnosis in the patients with neck symptoms was shorter than that in the patients with mainly nasal symptoms (Z=-2.078, P<0.05) or with mainly otologic symptoms (Z=-2.372, P<0.05).Conclusion Several nasopharyngeal malignancies may occur in children and adolescents, and usually have no specific clinical manifestations, among which patients with only nasal and/or otologic symptoms are especially easy to be misdiagnosed, and should be distinguished clinically.