BACKGROUND: Although myxoma is the most common form of benign cardiac tumor, which is a rare condition itself, less than 3-4% of cases are detected in the right ventricle (RV). The clinical presentations vary widely and are nonspecific, causing challenging diagnosis. Although rare, myxoma can coexist with infective endocarditis (IE). CASE REPORT: We report a rare case of right ventricular myxoma presenting with dyspnea and fever, which was later found to be complicated with concomitant tricuspid valve endocarditis during surgery as well as the performed surgical approach. CONCLUSION: RV myxoma is a rare entity requiring a high index of suspicion due to varying nonspecific presentations. Concomitant IE should be suspected in patients with persistent fever. Antibiotics and careful surgical approach are needed to prevent complications, including embolization.