Introduction This report describes a rare occurrence of corneal epithelial keratopathy associated with ribociclib, a cyclin-dependent kinase 4/6 (CDK4/6) inhibitor widely used in breast cancer treatment. Case report An 83-year-old female with a recent breast cancer diagnosis presented with blurred vision during the third cycle of ribociclib treatment. Ocular examination revealed corneal epitheliopathy and fluorescein staining findings. Detailed assessments and comparisons were made with existing literature, identifying a few reported cases of vortex keratopathy linked to ribociclib. To the best of our knowledge, this is the first reported case of epitheliopathy. Management and Outcome Since it decreased visual acuity, ribociclib treatment was discontinued in consultation with oncology. There was no regression in epithelopathy findings and visual acuity did not improve during follow-up visits. Discussion This report underscores the uncommon association between ribociclib and corneal epithelial keratopathy, emphasizing the need for comprehensive monitoring and collaboration between oncologists and ophthalmologists.