Ocular fungal infections annually affect more than one million individuals worldwide. They may occur as endogenous or exogenous fungal uveitis/ endophthalmitis. The latter appear after intraocular procedures, perforating ocular trauma or secondary to fungal keratitis. Although culturing is still the standard in the diagnosis of fungal endopthalmitis, more sensitive and specific and faster diagnostics tests include molecular tests; polymerase chain reaction and next generation sequencing. Also highly sensitive is the serum assessment of galactomannan and beta-D-glucan – two markers of fungal cell wall components. Treatment of fungal uveitis is difficult, mainly due to the limited permeability of antifungal drugs into intraocular fluids. In the treatment of fungal uveitis, systemic antifungal drugs are used, which in the case of vitreous involvement are combined with amphotericin B or voriconazole intravitreal injections. In cases with severe endophthalmitis and intravitreal abscess the vitrectomy is a treatment of choice.