Background <p>Scedosporium apiospermum endocarditis is an exceptionally rare condition, predominantly observed in immunocompromised individuals or those undergoing invasive interventions such as central venous catheterization or prosthetic cardiac valve implantation. Its occurrence in immunocompetent adults with native valves is even more uncommon. This case represents the first documented instance in China.</p> Case summary <p>This study presents the first documented case of Scedosporium apiospermum endocarditis in an immunocompetent male patient in China.</p> Conclusion <p>S. apiospermum endocarditis carries a grave prognosis. Among 18 reviewed articals, 19 patients succumbed to the infection, 7 of whom were diagnosed postmortem, precluding therapeutic intervention. Only one survivor was reported following implantable cardioverter-defibrillator (ICD) extraction and voriconazole treatment. These findings underscore the insidious onset, diagnostic challenges, frequent therapeutic delays, and extremely high mortality associated with this infection.</p>