Case ReportsSphenoid Dysplasia A Rare Presentation of Infantile MyofibromaKini, Ashwini M.D.*; Syed, Reema M.D.*; Compton, Christopher M.D.*; Hata, Jessica L. M.D.†; Ramasubramanian, Aparna M.D.*Author Information *Department of Ophthalmology and Visual Sciences, University of Louisville, and †Department of Pathology, Norton Children’s Hospital, Louisville, Kentucky, U.S.A. Accepted for publication October 29, 2017. Supported in part by an unrestricted institutional grant from Research to Prevent Blindness, New York, NY. The authors have no conflicts of interest to disclose. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s website (www.op-rs.com.). Address correspondence and reprint requests to Aparna Ramasubramanian, M.D., Department of Ophthalmology and Visual Sciences, University of Louisville, 301 E Muhammad Ali Blvd, Louisville, KY 40202. E-mail: firstname.lastname@example.org Ophthalmic Plastic & Reconstructive Surgery: March/April 2018 - Volume 34 - Issue 2 - p e65-e67 doi: 10.1097/IOP.0000000000001038 Buy SDC Metrics AbstractIn Brief The authors report a case of isolated congenital orbital myofibroma causing sphenoid dysplasia and presenting as global restriction of extraocular motility and ptosis in a neonate. Sphenoid wing dysplasia is most commonly associated neurofibromatosis 1 but this patient had no evidence of neurofibromatosis on clinical examination and genetic testing. Congenital orbital myofibroma can have secondary effects on bone and likely the lesion was present early in development leading to aplasia of the sphenoid bone. To the best of the authors’ knowledge, this is the first reported case of sphenoid wing aplasia secondary to congenital orbital myofibroma independent of neurofibromatosis 1. An infiltrating orbital myofibroma during fetal development can cause congenital sphenoid wing aplasia which may present as restrictive childhood strabismus and should prompt early imaging. © 2018 by The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc., All rights reserved.