The authors report an unusual case of Parry-Romberg syndrome (PRS). Magnetic resonance imaging and computed tomography scan of the craniofacial region and surgical correction of enophthalmos were performed. Results after the operative intervention included persistent palpebral edema and ecchymosis and transient choroid vasculitis of the right eye, highlighted with echography and fluorescein angiography. The fundus oculi examination showed retinal choroid folds. Immunological test results were weakly positive. The authors conclude enophthalmos, associated with right side hemi atrophy, and the transient choroids vasculitis support the diagnosis of PRS. Furthermore, it is suggested the case had an autoimmune etiology, rather than a hemi facial asymmetry caused by a facial trauma that occurred in puberty.