A 3-year-old girl presented with an opaque, globular protrusion in her right eye, characterized by a white, neovascularized cornea with a larger-than-normal diameter. Ultrasound imaging indicated an enlarged eye with an anteroposterior length of 29 mm, an abnormal anterior segment, and a normal flat retina and vitreous, with no intraocular mass detected. Computed tomography (CT) revealed an external pocket on the anterior aspect of the globe. Clinical and supportive findings led to the diagnosis of congenital corneal staphyloma. The patient underwent enucleation of the right eye, followed by the placement of a prosthesis. Histopathological examination of the surgical specimen confirmed the diagnosis of congenital corneal staphyloma.