Ameloblastomas are benign but locally invasive neoplasms which may grow to 
massive proportions and cause significant morbidity. Although some types of 
ameloblastoma can be treated predictably with aggressive surgical treatment, 
recurrent ameloblastoma and metastasising ameloblastoma are still difficult to 
treat. Recent studies have identified recurrent somatic and activating mutations 
in the mitogen-activated protein kinase (MAPK) and sonic hedgehog (SHH) 
signalling pathways in ameloblastoma. This development provided a possibility 
that molecular targeted therapies can be used as neoadjuvant treatment. In this 
review, we provide a summary of the latest WHO classification of ameloblastoma, 
the current understanding of genetic mutations and novel molecular targeted 
therapies arising from the recent developments.