Keratoglobus is a rare bilateral condition characterized by a globular protrusion of the cornea. The cornea remains transparent and shows a generalized thinning, most pronounced in the periphery. Most often keratoglobus is a congenital disease, but it may be secondarily acquired. This corneal pathology causes severe visual impairment: it induces extreme myopia, irregular astigmatism, and corneal scarring due to previous hydrops. Spectacle corrections are utilized as long as they provide adequate visual function. Surgery is performed when visual needs are no longer satisfied. However, surgical management of this disease is difficult because of the extreme thinness of the entire cornea. Penetrating keratoplasty is one of the first surgical approaches to have been tested, but it has been partly renounced in favor of more complex procedures. Optimal surgical treatment for keratoglobus remains unknown because of the scarcity of reported surgical results.