Aspergillus infective endocarditis (ASIE) is a very rare disease that carries an extremely poor prognosis. We report a case of ASIE successfully treated by a tricuspid valve replacement and administration of an antifungal drugs. The patient was a 69-year-old man who was taking steroids for an autoimmune disease and was admitted to our hospital because of a persistent fever of 39°C. As chest CT showed infiltrative shadows in both lung fields and the aspergillus antigen was detected in the blood, we diagnosed invasive pulmonary aspergillosis (IPA), and initiated administration of micafungin sodium (MCFG). Later, the patient's heart failure worsened, and echocardiography revealed vegetation measuring 8 mm in diameter in the anterior cusp of the tricuspid valve. As this indicated a definitive diagnosis of ASIE, administration of voriconazole (VRCZ) was initiated. However, the vegetation grew into a movable wart measuring 20 mm in diameter within a week, based on which a diagnosis of drug-resistant ASIE was made, and surgery was considered indicated. Very large vegetations were found in the anterior cusp of the tricuspid valve, anterior papillary muscle, the tendinous chord of the medial papillary muscle and the trabeculae carneae of the right ventricle. Based on the findings, it was judged that tricuspid annuloplasty was impossible and tricuspid valve replacement was performed using a biological valve. As to the antifungal medication, long-term administration of VRCZ and MCFG was continued. The patient followed a favorable course and was discharged from the hospital on the 220th day. The patient aking lifelong VRCZ and has shown no evidence of recurrence of the ASIE. To improve the prognosis of ASIE, rapid and radical surgical resection of the vegetations and appropriate administration of antifungal drugs are important.