Ameloblastomas are generally considered to be benign tumors of odontogenic epithelial origin with high local recurrence. Rarely ameloblastomas exhibit malignant behavior with development of metastases. In this report, we present a 19-year-old woman with ameloblastoma in the right ascending ramus and multiple recurrences. Eleven years after first therapy, ameloblastoma metastasized to ipsilateral submandibular lymph node. We also review literature about cause, treatment and work-up of malignant ameloblastoma.