Ameloblastomas are histologically benign tumors derived from the odontogenic 
apparatus. Although these tumors are locally invasive, they rarely invade the 
paranasal sinuses, orbits, or intracranial cavity, and, thus, they rarely 
produce ophthalmologic signs and symptoms. In this report, we describe the 
neuro-ophthalmologic features of three patients with chronically aggressive 
ameloblastoma. Two of the patients developed a progressive and recurrent orbital 
apex and cavernous sinus syndromes. One of these patients is, to our knowledge, 
the first patient described with orbital and cavernous simus involvement by an 
ameloblastoma initially arising in the mandible. The other is only the second 
case described with bilateral orbital involvement. The third patient in this 
series developed a trigeminal sensory neuropathy as the only sign of the tumor. 
Although ameloblastomas are benign, slowly growing tumors, they may, often over 
a long period of time, cause significant neuro-ophthalmologic and orbital 
manifestutions that can only be partially ameliorated by surgery.