Background: Cutaneous stigmata (Janeway lesion and splinter haemorrhages) are rarely seen in acute infective endocarditis (IE) and serve as an important clue to its early diagnosis and prompt treatment. Case Report: A 20-year-old male presented with intermittent, high grade fever of 2 weeks duration, palpitation and worsening dyspnea. On examination, Janeway lesions and splinter haemorrhages were noted. Echocardiogram revealed large, mobile echogenic mass attached to ventricular surface of right coronary cusps which was perforating the valve causing acute severe aortic leak. He underwent successful aortic valve replacement. Conclusion: These cutaneous lesions may be rarely seen in acute IE and serve as an important clue to IE as prognosis is grave because of high morbidity and mortality and therefore, early diagnosis and prompt treatment may be life saving.