Although rare, necrotizing skin and soft tissue infections can be devastating infections that are difficult to diagnose and challenging to manage. Clinical presentation is often insidious, and a low index of suspicion is critical. Various diagnostic tools, such as scoring systems or imaging techniques, have been introduced, but none is convincingly superior to sound clinical judgment. Early diagnosis allows early adequate therapy that includes antibiotic therapy, critical care support, specific interventions such as intravenous immunoglobulin in selected patients and, most importantly, early source control. Empirical antibiotic therapy should cover a broad range of both Gram-negative and Gram-positive aerobic and anaerobic microorganisms, and clindamycin is recommended when group A Streptococcus is a suspected pathogen.