Aspergillus infectious endocarditis (IE) is a rare cause of culture-negative endocarditis. The main risk factors are severe immunosuppression and prosthetic heart valve. We describe a case of Aspergillus fumigatus IE on a native mitral valve in a patient with autoimmune hepatitis in remission while on low dose corticosteroids. The case is unique due to the patients' low initial risk for invasive fungal disease, its clinical presentation and successful management with emergency surgery and antifungal therapy. After literature review we have not found a similar case report. The patient presented with right-sided eyesight deterioration due to endophthalmitis. Vitrectomy was performed and Aspergillus fumigatus grew on culture. IE on a native mitral valve was confirmed with echocardiography. The patient developed signs of acute heart failure soon after hospital admission and was diagnosed with several septic emboli (kidney, spleen, thumb, right common femoral artery). He was initially treated with surgical valve replacement, dual antifungal therapy with liposomal amphotericin B (LAmB) and voriconazole and vitrectomy, including intravitreal amphotericin B application. Long-term triazole therapy was not possible due to hepatotoxicity. The patient was maintained on intermittent LAmB for 12 months and is without signs of recurrence ten months after therapy was discontinued. Aspergillus can cause invasive infection in patients with autoimmune hepatitis on low dose corticosteroids. Early diagnosis followed by emergency surgical valve replacement and systemic antifungal therapy can improve prognosis. Additional studies are needed to evaluate alternative methods and duration of antimicrobial therapy following Aspergillus IE.