Aerococcus-related infective endocarditis is a rare form of endocarditis with a high mortality. Most cases have associated genitourinary system pathology. Aerococcus urinae is a Gram-positive coccus that is arranged in clusters resembling Staphylococcus, but its growth characteristics and colonial morphology are closer to that of an alpha-hemolytic Streptococcus. This organism may initially be dismissed as a contaminant in clinical cultures from nonsterile sites. We report a representative case and review the literature concerning this unusual case of infective endocarditis.