Objective To explore the clinical diagnosis and treatment of non-small cell lung cancer with tonsil metastasis. Methods The data of non-small cell lung cancer with tonsil metastasis was analyzed retrospectively. The clinical features, diagnosis and treatment of the disease were discussed by reviewing the relevant literatures. Results The patient was diagnosed with non-small cell lung cancer by fiberoptic bronchoscopy. The right tonsil and tumor resection were performed due to a huge pharyngeal mass that interfered with breathing and swallowing. The patient underwent right tonsillectomy and tumor resection due to the obstruction of breathing and swallowing because of huge pharyngeal tumor. The sample of the patient by postoperative pathology was confirmed for metastatic non-small cell lung cancer. After the operation, the patient was treated with radiotherapy and chemotherapy, but the left tonsil and brain metastases occurred quickly, who died 5 months later. Conclusions It is rare for bilateral tonsil metastasis of non-small cell lung cancer with rapid progress and poor prognosis. Surgical treatment can relieve respiratory obstruction. Clinicians should not only consider the primary tumor, but also be alert to the possibility of metastatic cancer when the tonsils of the patients are abnormally enlarged. For patients with lung cancer, oropharyngeal examination should also be routine to avoid missed diagnosis.