Background <p>Infective endocarditis is a rare but serious infection of the heart valves, with various clinical manifestations. Diagnosis relies on the modified Duke criteria, in which Janeway lesions can be an important diagnostic feature.</p> Case presentation <p>We report a case of a Middle Eastern male patient in his mid-60s with history of ischemic heart disease, diabetes mellitus, chronic kidney disease, and hypertension who presented with respiratory symptoms and extensive skin lesions. A skin biopsy confirmed the lesions as Janeway lesions, assisting in the diagnosis of infective endocarditis caused by <i>Enterobacter</i> species. Despite aggressive antimicrobial therapy, the patient’s clinical condition failed to improve, highlighting the challenges in managing infections caused by this organism.</p> Conclusion <p>This case highlights the importance of recognizing atypical manifestations of infective endocarditis and utilizing diagnostic tools, such as skin biopsy, for accurate diagnosis. It emphasizes the need for effective management strategies in extended cases caused by uncommon organisms such as <i>Enterobacter</i>.</p>