A 4-month-old female infant presented to the Strabismus and Pediatric Ophthalmology Center in Tianjin Medical University Eye Hospital with sudden burst of a pink neoplasm in the right cornea and blepharospasm for one day. Previously, the patient had already been diagnosed with corneal dermoid in another hospital on postnatal day 7. She could fix and follow small toys with her left eye while patched the right eye, but cried loudly when the left eye was covered. A brownish-black hemispherical neoplasm (diameter, 4.5 mm) was observed in the center of the right cornea with surrounding stromatic edema and extremely flat anterior chamber. In addition, a mild ciliary injection was observed in the right eye, with a much larger cornea (diameter, 12.0 mm). The anterior synechia of the iris was obviously beneath the lesion of the cornea, while the other internal structures of the right eye were not clearly visualized. The cornea of the left eye (diameter, 11.0 mm) was clear and no abnormality was found. Color Doppler ultrasonography was performed for the right eye under sedation, which revealed that the eyeball was intact, while a crumby lesion that linked the cornea and the iris was observed in front of the crystalline lens; however, the vitreous cavity was clear and no abnormality was found in the posterior segment. The infant was the second child delivered normally by the mother at full term. General physical examination of the infant revealed no other noticeable abnormality. However, the mother had a history of repeated upper respiratory tract infection during the pregnancy. Tobramycin eye drops were used four times a day to prevent infection in the right eye of the infant after admission. The corneal neoplasm in the right eye turned red on the next day; however, the volume of the lesion was apparently unchanged. The infant calmed down, and the symptoms of eyelid irritation disappeared. Initially, the baby was suspected to be suffering from corneal perforation, Peters’ abnormality, and congenital glaucoma. A surgical exploration on the right eye was performed under general anesthesia on day 4 after admission. During the operation, a red, firm, solid mass with dilated small vessels was found on the surface. No weakening or perforation was observed in the cornea. The protuberant part of the mass was excised, revealing the wound with a boundary that clearly differentiated it from the surrounding corneal tissues. The residual mass had a spiral-shaped presentation, while no distinct pigment tissue was found. Anterior chamber paracentesis was performed at 11 o'clock position of the limbus, and only a little aqueous fluid flowed out. Considering the possibility of pupillary block caused by anterior synechia, peripheral iridotomy was also performed. TobraDex eye ointment was applied to the eye, followed by eye patching. The mass was sent for pathological examination postoperatively. Conventional hematoxylin & eosin staining revealed multilayers of well-differentiated mature squamous epithelia on the surface of the mass; however, the cells on basal layer were well arranged, without atypia. Numerous fibroblast-like cells were observed, with a small amount of mature collagen fibers and blood vessels. The tumor cells were spindle-shaped, and arranged irregularly. These cells were eosinophilic and full of cytoplasm, and the nuclei were oval or fusiform, and lightly dyed without obvious atypia or mitosis. Some cells showed slender cytoplasmic protuberances that connected with collagen fibers. In addition, scattered brown pigment particles were observed among the tumor cells. Immunohistochemical staining showed strong expression of vimentin and smooth muscle actin (SMA) in the tumor cells, while desmin was only partially expressed; however, no sign of S-100 protein or CD34 expression was found in these cells. In contrast, for vascular endothelial cells in the tumor tissues, CD34 was found to be positively expressed. Therefore, the pathological diagnosis for the lesion was myofibroblastoma in the cornea of the right eye. The parents were well informed and the baby was taken back to have a check every 3 months. At the 12-month follow-up, a scar was found in the cornea of the right eye, while the diameter of the cornea was not increased, and the intraocular pressure was normal. Color Doppler ultrasonography and magnetic resonance imaging (MRI) were performed for the right eye, which showed that the mass was restricted but connected with the cornea and the iris; the back boundary of the mass was in front of the crystalline lens, and no growth of the mass was observed.