Ameloblastic carcinoma (AC) is a very rare odontogenic malignancy. Primary AC is more common in maxilla, while secondary AC is more common in mandible. Histologically, it is an ameloblastoma with features of cancer. Presence of metastasis is not necessary to establish the diagnosis. This tumor usually metastasises in bone or lungs. We report a case of aggressive secondary AC of mandible with lung metastasis. There are few cases in medical literature discussing management of AC with lung metastasis. In this case, a 42-year woman, who was diagnosed with ameloblastoma of right mandible, was treated with right hemi-mandibulectomy. However, 10 years later, the patient developed AC with pulmonary metastasis. Patient received six cycles of carboplatin-paclitaxel-based regimen. There was noted a partial response to the therapy. However, the patient went into respiratory failure, although she was provided adequate analgesic control as part of end-of-life care. Further, oncological or surgical management was not possible as patient had poor performance status.