Ameloblastoma of the mandible is a rare odontogenic tumour that rarely metastasizes. We report a patient with a slowly progressing ameloblastoma of the mandible diagnosed at the age of seven and resected multiple times due to tumour recurrence. Multiple pulmonary metastatic nodules were resected at thoracotomy 27 years after the initial diagnosis; however, further pulmonary disease was discovered. The patient was admitted with chest pain due to pulmonary metastases 45 years after the initial diagnosis. The metastases were intraluminal and could be attributed to tumour cell aspiration during the surgical procedures on the mandible. The patient also suffered from hypercalcaemia which was attributed to a parathormone-like substance secreted by the tumour.