Objective:To study the clinical manifestations and treatment of the primary cervical tracheal malig-nant tumor of children. Method:Five cases diagnosed as primary tracheal malignant tumor with fibrolaryngoscopy, CT scan and pathology were retrospectively analyzed and reviewed. Extraction of malignant tumor was performed with tracheoscope and nasal endoscope after emergent tracheotomy below the tumor in 4 cases, and tracheotomy after intubation in 1 case. After operation, 4 children received radiotherapy, and 1 case received laser therapy add radiotherapy. Result:Five cases were followed up for three to eight years, and no recurrence was found. Conclu-sion: There was no specific manifestation in early tracheal malignant tumor, but it was critical and emergent during symptoms appear. Imaging examination was a very invaluable dignosis method. Optimal and effective therapeutic protocol is tracheostomy under local anesthesia to keep upper airway patency followed by surgical extraction and radiotherapy. It was not appropriate to have tracheal wall resection for children.