Endogenous fungal endophthalmitis has increased in the past half-century because of the advent of antibiotics and indwelling catheters. The disease process can produce highly suggestive, though nonpathognomonic, ocular signs that assist the clinician in reaching a diagnosis. Intraocular inflammation, especially if it is granulomatous in nature in a patient with one or more of the risk factors already discussed, should raise the suspicion of fungal endophthalmitis. If a diagnosis remains elusive, vitreous biopsy is indicated for proper identification of a fungal organism. Although a particular therapeutic regimen has not yet been prospectively established, compelling arguments can be made to treat nearly all patients with endogenous fungal endophthalmitis with a systemic antifungal such as amphotericin B or fluconazole. A possible exception includes an IVDA-related endophthalmitis in a patient with negative blood cultures and without other evidence of fungemia. Vitrectomy and intravitreal amphotericin B (with or without intravitreal corticosteroid) should be considered in cases of endogenous fungal endophthalmitis in which there is substantial vitreous involvement, and also in cases in which there is clear progression of disease despite initial therapy with an appropriate systemic antifungal agent. Prompt therapy following early diagnosis will help reduce significant visual loss in all forms of fungal endophthalmitis.