Background Fungal endocarditis is a rare form of endocarditis accounting for less than 2% of all cases. Common offending pathogens include: Candida and Aspergillus. Cryptococcal endocarditis is extremely rare with only ten prior cases reported. To our knowledge, only one prior case of AICD-associated cryptococcal endocarditis was described in the literature. Case A 57-year-old female patient presented to the emergency department with headache, fatigue, and a near-syncope event. Her past medical history includes ischemic cardiomyopathy with AICD implantation and vasculitis (on prednisone and cyclophosphamide). Examination was unremarkable. CT scan of the head was negative for acute intracranial process. In lieu of persistent headache, lumbar puncture (LP) was performed revealing lymphocytic pleocytosis. Transthoracic and transesophageal echocardiography revealed vegetations on the ventricular lead of the defibrillator measuring 2.0 × 0.67 cm. Decision-making Patient was initially started on IV vancomycin before adding flucanzole empirically to the regimen given her immunosuppressed status and lymphocytic pleocytosis on LP. On the 4th day of admission, two sets of blood cultures grew Cryptococcal Neoformans. Hence, patient was switched into amphotericin B and flucytosine for 2 weeks before resuming fluconazole. Concomitantly, a decision was made to remove the AICD device along with leads to achieve source control. Despite lack of data on proper duration of treatment, high-dose fluconazole was continued for a total of 4 weeks (one year is typically recommended in cryptococcal meningitis). Patient improved gradually before achieving complete recovery, without relapse. Conclusion Cryptococcal endocarditis is an extremely rare event with no prior standardized treatment protocol established. Hence, treatment duration with antifungals need to be individualized. Following an initial inductive phase, prolonged suppressive therapy with Fluconazole might be warranted to prevent recurrence, especially in immunocompromised patients. AICD-related infections mandate device removal. Surgical intervention has no clear indication but should be considered
