Abstract
Unicystic Ameloblastoma (UA) is a rare variant of ameloblastoma which is an odontogenic epithelial neoplasm, typically affecting mandibular ramus. Maxillary ameloblastoma is a rare entity with a more disastrous consequence. Although extremely rare, their highly recurrent and locally aggressive behavior can lead to invasion of vital structures surrounding maxilla (orbit, cranium) even after several years of conservative surgical management (limited resection, curettage). We report a case of 16-year-old girl presenting with proptosis of left eye, UA left maxilla, who was treated initially with limited resection (enucleation) and curettage and the lesion recurred after two years with a more aggressive behavior, causing destruction floor of orbit. To this date there are only 23 documented cases of orbital invasion and only three of the reports are in ophthalmic literature. The ophthalmologists need to be aware of this type of rare lesion presenting as proptosis.