In this manuscript we describe the first association in the literature between Abiotrophia defectiva endocarditis and the hemophagocytic syndrome. There are multiple important clinical points of information that must be highlighted from this case. A. defectiva is an aggressive organism with a high level of resistance to antibiotic pharmacotherapy with a high predilection for embolic complications and valvular destruction despite treatment with sensitive antibiotics. A. defectiva endocarditis has not been previously associated with the hemophagocytic syndrome. However, this case highlights the serious hematological complications that can occur with this dangerous bacterial pathogen.