Among clinically isolated β-hemolytic streptococci, Streptococcus pyogenes and S. agalactiae were considered the main pathogens in humans until recently. In 1996, S. dysgalactiae subsp. equisimilis (SDSE) was proposed as a novel taxon among human-derived streptococcal isolates. SDSE has Lancefield group C or G antigens, exhibits strong β-hemolysis, and exerts streptokinase activity upon human plasminogen and proteolytic activity upon human fibrin. Similarly to group A streptococci, SDSE possesses virulence factors including M protein, streptolysin O, streptolysin S, streptokinase, hyaluronidase, C5a peptidase, and others. SDSE may exist among the normal flora of the skin, oropharynx, and gastrointestinal and genitourinary tracts. In the twenty-first century, invasive SDSE infection (i.e., cellulitis, urosepsis, and pneumonia) leading to various disseminated diseases is being diagnosed increasingly in Japan, elsewhere in Asia, in Europe, and in America. Particularly, among elderly patients, these invasive diseases are encountered increasingly in Japanese hospital emergency departments. Analysis of the part of the emm gene encoding the amino acid sequence at the N-terminal end of the M protein is used to determine the molecular epidemiology of SDSE. The distribution of emm types from patients with invasive or noninvasive infections differs between surveillance results from different countries. In this review, we summarize the characteristics of phenotypes and virulence factors in SDSE strains; the review also focuses on emerging SDSE infectious disease and future vaccination research.