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Fibrous dysplasia was first described by Lichtenstein in 1938 as a disorder characterized by progressive replacement of normal bone elements by fibrous tissue. It is a bone tumor that, although benign, has the potential to cause significant cosmetic and functional disturbance, particularly in the craniofacial skeleton. Its management poses significant challenges to the surgeon. Its compression of the optic nerve with resulting visual impairment is especially alarming. Over the years, we have gained a better understanding of its etiology, clinical behavior, and both surgical and non-surgical treatments. Its characteristics, under various imaging modalities, have been thoroughly described in recent years. These developments have taken place with the goal of optimizing treatment of those who suffer from this disease. However, the role of prophylactic optic nerve decompression in cases of optic canal involvement has recently been challenged: the results of a few recent studies have raised questions regarding its role. Further studies would be required to assess its value.