Nevus of Ota with midface tumors and a vanishing orbital mass

Nevus of Ota, also known as oculodermal melanocytosis, is a benign melanocytic lesion that develops along the distribution of the V1 and V2 branches of the trigeminal nerve. Prior reports have described the typical imaging and clinical features of nevus of Ota. We present a rare case of a 31 year-old female with midface tumors and presumed hemorrhage into an orbital lesion in the setting of nevus of Ota resulting in acute loss of vision.