We review the clinical, histopathological, and ultrastructural findings and DNA phenotyping of a patient with Avellino corneal dystrophy exacerbated by laser in situ keratomileusis. The findings are reported and interpreted in the context of a literature review. The case highlights the possible difficulty of recognizing subtle dystrophic findings, as well as the importance of avoiding refractive surgical intervention in patients with Avellino corneal dystrophy to avoid exacerbation of dystrophic deposits in the cornea and subsequent reduction in vision.