Histopathologic examination revealed massive laminated orthokeratosis with papillomatous acanthosis and innumerable fungal hyphae in the cornified layer highlighted with PAS. Culture grew Trichophyton rubrum. Connexin-26 gene (GJB2) sequencing identified a heterozygous mutation (c.148G>A). The patient was evaluated by the ophthalmology department and diagnosed as having severe open-angle glaucoma and diffuse keratitis of the cornea. Both retinas were nonviable, and she was not a candidate for a repeated corneal transplant. Daily warm water baths with diluted vinegar were begun. A compound containing 6% lactic acid and 10% urea in Aquaphor ointment (Beiersdorf AG) was applied twice daily. The patient was treated with oral fluconazole, 300 mg, once weekly for 4 weeks. She experienced a marked improvement in the hyperkeratosis of her trunk and scalp within 2 weeks.