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To report an unusual case of isolated Acremonium eumycetoma presenting as a protuberant mass over the cornea.Case report and literature review.A 55-year-old male patient referred to our center with a case of perforated corneal ulcer with uveal tissue prolapse was examined in the casualty department and found to have central melt, approximately 8 mm, along with suspected uveal tissue prolapse. A provisional diagnosis of sloughed corneal ulcer with uveal prolapse was made along with differential diagnoses of fungal ball and infected foreign body granuloma. Tectonic penetrating keratoplasty under general anesthesia was planned. Intraoperatively, the suspected uveal (brown colored) tissue was found to be an epicorneal mass growing over an intact and infiltrated cornea. Histopathological and microbiological analysis of the epicorneal mass and host cornea revealed it to be a fungal ball (mass full of septate hyphae) with growth of Acremonium species on culture. The patient was administered topical and oral antifungal agents postoperatively, in addition to topical antibiotics and cycloplegics.Isolated corneal Acremonium eumycetoma masquerading as a perforated corneal ulcer with prolapsed uveal tissue is a rare entity. Surgical intervention and appropriate antimicrobial therapy are key to successful outcome.