Introduction: Fungal Endocarditis is rare and fatal. Most prevalent in immunosuppressed and Intravenous Drug Abusers. Candida and Aspergillus species are most common etiologic Fungi. Case presentation: A Seventeen-year-old male with known Hypertensive, Seizures and Alport Syndrome of Stage V Chronic Kidney Disease, admitted with history of fever and chills for 2weeks. On regular hemodialysis via right Internal Jugular Vein Permcath, inserted 6 months ago. Clinical examination was unremarkable except for tachycardia and hypotension. Serial blood cultures were negative for any growth. But 2D Echocardiography confirms the presence of mobile vegetative mass attached to the Tricuspid Valve (TV), which extends and then obstructs the Right Ventricular Outlet Tract (RVOT). With worsening symptoms and failed initial management, he was admitted first time and underwent removable of Permcath, TV Vegetectomyand then Pericardial patch augmentation of Septal Tricuspid Leaflet and Alferi type of TV repair. Both Permcath tip and excised mass were sent for Histopathological Examination (HPE), confirming Fungal infection with Magnusiomyces Capitatus, which belongs to Blastoschizomyces capitatus. Responds to Inj. LoposomalAmhotericin B and Voroconazole. But within few weeks, he developed Severe Tricuspid Regurgitation and Right heart failure with gross Ascitis. Then readmitted for a second time and underwent immediate symptomatic relief by Ascitic tapping followed by TV Replacement with 31 mm of St. JUDE Mechanical Valve. Perioperative management was challenging and stabilized gradually and involves a multispecialty team approach. Discussion: Fungal endocarditis is a serious condition. Combined aggressive Medical and Surgical therapy will have a better outcome