The spectrum of illnesses caused by group A streptococcus (GAS) includes invasive infections, noninvasive infections, and noninfectious complications. Increasingly virulent infections associated with high morbidity and mortality have been observed since the late 1980s and continue to be prevalent in North America and worldwide. Penicillin remains the therapy of choice, with the addition of clindamycin recommended in high risk cases. Early recognition of GAS as the cause of these serious clinical syndromes is critical for timely administration of appropriate therapy. In this review, the pathophysiology, clinical manifestations, and treatment of invasive GAS infections are discussed.