Ameloblastomas are rare odontogenic epithelial tumors that occur mainly in the mandible. Despite their benign histologicappearance, they are locally aggressive with a high recurrence rate. However, a metastasizing ameloblastomahas been rarely reported. According to the current World Health Organization classification system, thedefinitive diagnosis of metastasizing ameloblastoma can only be carried out in retrospect, after the event ofmetastasis. This case report describes a patient with metastatic pulmonary ameloblastoma, 17 years after the surgicalexcision of an odontogenic tumor, preoperatively misdiagnosed as primary squamous cell carcinoma.