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To summarize the clinical, radiographic, and intraoperative characteristics of isolated neurofibromas of the orbit (INFO) in the literature and report a case of INFO.Case report and a systematic review of the literature.A total of 45 patients were identified from 18 previous studies. There was no sex predilection and mean ages were between 32.3 and 40.0 years with a standard deviation of 9.8 to 19.5 years, median age was 32 to 38 years with a range of 1.5 to 82 years. On CT imaging, INFO reveals homogeneous precontrast radiodensity similar to that of extraocular muscles. On MR imaging, INFO appears hypointense to orbital fat and isointense to brain on precontrast T1-weighted images. On T2-weighted images, they have been described as being predominantly hyperintense with possible hypointense intralesional islands. To the best of our knowledge, our case identifies the youngest patient with the myxoid histologic variant of INFO.Patients with orbital neurofibromas should be evaluated for the presence of systemic neurofibromatosis, and the plexiform variant is a commonly associated subtype. The myxoid variant can be part of a lesser known condition called NAME syndrome (nevi, atrial myxoma, myxoid neurofibroma, and ephelides) which may warrant echocardiography in patients to evaluate for associated cardiac tumors.