Edenia gomezpompae, isolated from a patient suffering from keratitis, who was an agricultural worker with a 4-year history of diabetes mellitus, is described. Fungal hyphae were detected in corneal scrapings after microscopic examination and repeated culture. Isolates were identified by morphology and by sequencing the internal transcribed spacer region of ribosomal DNA. Chlamydospore characteristics of E. gomezpompae are described for the first time. The in vitro antifungal susceptibilities of the isolate were tested using five antifungal agents. With the exception of fluconazole, the other drugs (natamycin, amphotericin B, itraconazole, and voriconazole) assayed were highly effective against this fungus. The keratitis was cured after debridement of the corneal ulcer and treatment with 5 % natamycin. After 3 months, when the patient was reexamined, the corneal ulcer showed signs of healing, with no recurrence. To our knowledge, this is the first reported case of keratitis caused by E. gomezpompae, as well as being the first known case of disease in humans caused by this species. Keywords Edenia gomezpompae * Mycotic keratitis * Chlamydospores * Antifungal susceptibility testing