Ameloblastoma is a benign odontogenic tumor which undergoes malignant 
transformation to ameloblastic carcinoma. However, rarely it metastasizes 
without undergoing cytological malignant changes, an entity referred to as 
Metastasizing Ameloblastoma (MA). Through this study, we aimed to review cases 
of MA reported since 2000 to explore the impact of clinico-demographic variables 
on its prognosis. Based on PRISMA guidelines, a review of relevant literature 
from PubMed/Medline, Science Direct and Cochrane database was performed from 
January 2000 to March 2019. A total of 65 cases were considered for further 
evaluation as per predefined inclusion and exclusion criteria. Results showed 
that lungs followed by lymph nodes were the most common sites for benign 
metastatic deposits. Multiple recurrences and inadequate surgical removal 
increase the probability of distant metastatic spread. Despite having benign 
cytological features, tumor recurrence and metastasis were associated with an 
unfavorable clinical outcome in MA.