PURPOSE To describe a patient with Exophiala jeanselmei keratitis and to review the prior cases reported in the literature. METHODS We report one patient with keratitis after remote injury and chronic steroid use and review the six prior reported cases. RESULTS Culture plates from corneal scraping revealed growth of the dematiaceous fungi, E. jeanselmei, a rare causative organism of ocular infection. The patient underwent therapy with topical and intracameral antifungals and subsequently required a corneal transplant. Biopsy of the donor graft confirmed the diagnosis of E. jeanselmei. This is the first reported case to use intracameral antifungal agents and the first biopsy proven case. Half of reported cases experienced associated trauma, and severity was generally related to delay in diagnosis. CONCLUSIONS Trauma and chronic topical steroid use contributed to the development of severe keratitis in this patient. Patients on chronic steroids should be monitored closely. Topical, subconjunctival, and intracameral antifungals have all been effective in treating this pathogen. If diagnosed and treated early, E. jeanselmei keratitis can have a good visual outcome.