Background: We describe a rare case of metastatic ameloblastoma in a 68-year-old Chinese woman diagnosed with ameloblastoma of the right mandible in 1974. Case presentation: Despite radical resection, she experienced repeated local recurrences, including asymptomatic bilateral pulmonary nodules, cough, and hemoptysis diagnosed as non-malignant inflammatory cell infiltration. The patient developed an aggravated cough, palpitations, and dyspnea and was admitted to our respiratory department in September 2018. Chest computed tomography revealed multiple bilateral lung nodules, masses, and soft tissue opacities. She was diagnosed with advanced metastatic ameloblastoma in bilateral lungs via bronchoscopic biopsy, with a misdiagnosis time of 32 years. Because her systemic condition was poor, systemic chemotherapy and radiotherapy were not recommended. Dyspnea significantly improved after interventional bronchoscopic therapy and tumor electroexcision; she died of respiratory failure 1 year after diagnosis. Conclusions: Clinicians should carefully explore the medical histories of patients with surgical histories of ameloblastoma and subsequent unexplained pulmonary metastatic tumors to avoid misdiagnosis.