A case of streptococcal toxic shock like syndrome occurring in a 44-year-old previously healthy Japanese male is reported. He initially had a sore throat, low grade fever, diarrhea and mild pains in a lower extremities. Shortly thereafter, he rapidly developed a high fever, profound hypotension, multifocal epidermal necrosis, and sever purulent fasciitis and myositis in both lower extremities, which required above knee amputation of both legs. He later developed disseminated intravascular coagulopathy, adult respiratory distress syndrome, acute renal failure, coma and necrotizing inflammation of both arms and external genitalia despite treatment. He died on the 13th hospital day. Streptococcus pyogenes was isolated from the necrotic muscles and right knee joint. The organism was typed as M3, T3, was sensitive to penicillins, and was found to be producing streptococcal pyogenic exotoxin A in vitro. This is the first case report of streptococcal toxic shock like syndrome in Japan.