Sphyngomonas paucimobilis (S. paucimobilis) is a low-pathogenicity, gram-negative bacilli (GNB) that are previously known as an opportunist microorganism. Recent studies have shown that S. paucimobilis is an emerging pathogen causing various infections. Multidrug-resistant GNB has emerged as a major clinical and therapeutic dilemma in various hospital-associated infections. Although rare, S. paucimobilis could be associated with infective endocarditis (IE). Prosthetic valve endocarditis (PVE) is the most severe type of IE, which has high mortality rates despite diagnostic and treatment advances. We report a fatal case of early PVE associated with multidrug-resistant (MDR) - S. paucimobilis complicated with perivalvular abscess, complete heart block, valve detachment, and septic arthritis.