Background: Keratoglobus is a rare noninflammatory corneal disorder characterized by diffuse corneal thinning and globular protrusion of the cornea. Surgical management of keratoglobus is challenging and the standard method has not yet been defined. Aim: To present the role of large penetrating keratoplasty (PK) in the management of keratoglobus. Case Presentation: A 29-year-old male patient with bilateral keratoglobus presented with acute corneal hydrops in his right eye following extensive Descemet's membrane rupture, with a visual acuity in this eye limited to hand movement. Peripheral cornea was extremely thin and blue sclera was present. Acute hydrops was managed conservatively at this stage and two months later large PK was performed in the right eye using 9.5 mm diameter graft over a 9 mm patient's cornea trephination. Minor aqueous leakage was seen on the first postoperative day, managed with 2 more interrupted 10.0 nylon sutures. No further complications were noticed and postoperative course was uneventful. Fifteen months postoperatively the graft was clear and best corrected visual acuity was 20/60. Conclusions: Large penetrating keratoplasty has an important role in the management of keratoglobus, in cases where peripheral tuck-in lamellar keratoplasty or epikeratoplasty present serious intraoperative difficulties in host lamellar dissection and in stabilizing the graft due to extensive peripheral corneal and scleral thinning.