Aim: To report an unusual case of a newly diagnosed bilateral superior keratoconus in an elderly patient. Background: We report a case of bilateral superior keratoconus in a 64-year-old woman who was diagnosed after having presented with hydrops in one eye. Case description: Uncorrected visual acuity was counting fingers from 3 and 1 meter for the right and left eyes, respectively. A superiorly located deep stromal scar and chronic corneal edema consistent with a diagnosis of corneal hydrops were noted for the left eye. Although the cornea was clear, there was superior corneal protrusion in the right eye. Pentacam and Orbscan demonstrated overlapping focal corneal thinning, increased back elevation, and steepening in the superior cornea. Anterior segment optical coherence tomography (AS-OCT) showed stromal and epithelial thinning in the superior cornea and significant compensatory epithelial thickening in the opposing quadrant. In light of these findings, a diagnosis of superior keratoconus was established. Conclusion: Superior keratoconus is a rare condition. Meticulous evaluation of corneal tomography and epithelial thickness profile is crucial for localizing the superior cone in keratoconus. Clinical significance: This is the first case report demonstrating epithelial thickness profile in a superior keratoconus case.