Ameloblastic carcinoma is an extremely rare malignancy arising from odontogenic epithelium. The present work aimed to report clinical, histopathological, surgical and treatment-related details of four patients identified in our hospital database. We also present the disease behavior on follow-up of these patients. All patients underwent an adequate initial assessment and were treated with curative intent. The study population has a median follow-up of 68.5 months with no disease-related mortality. Literature suggests ameloblastic carcinoma to be chemo resistant and radio resistant. Surgical resection with wide margins is the standard of care. We also present a case of solitary lung lesion identified on follow-up imaging and addressed surgically. As surgery is the mainstay treatment in ameloblastic carcinoma, pulmonary metastasectomy is a valid tool in treating patients with isolated metastases.