Introduction: Ameloblastoma is a neoplasm usually found in the maxilla and mandible with progressive intraosseous growth and a tendency to local recurrence. Malignant or metastatic ameloblastoma is one of its rare variants; a histopathological study is required for diagnosis confirmation. The common sites of dissemination are the lungs and cervical lymph nodes. The treatment is surgical and radiotherapy or chemotherapy are palliative options.Case report: A case of malignant ameloblastoma was reported in a 39-year-old man with metastasis to the scalp and multiple local recurrences. The patient required mandibular osteotomy, multiple local resections, partial maxillary resection, free flap reconstruction, local scalp resection and free flap coverage. Due to a new relapse that could not be treated with a surgical approach, radiotherapy was ordered.Discussion: The site most frequently affected by malignant ameloblastoma due to hematogenous dissemination is the lung. This paper reports the case of a patient with compromised scalp. To date, according to a search conducted, only one case report about this type of metastatic neoplasm has been published.Conclusion: Histopathological diagnosis of ameloblastoma associated with surgical resection of the primary tumor and the metastatic tumor is the only approach that can offer disease-free survival.