Objective Clostridial sepsis has a very poor prognosis, owing to the life-threatening combination of shock and acute massive hemolysis. No papers have described the clinical features of clostridial sepsis cases in Japan. Therefore, we retrospectively examined the clinical features of patients with systemic inflammatory response syndrome (SIRS) from whose blood cultures Clostridium perfringens was isolated. Subjects and Materials Blood samples were obtained from SIRS patients and cultured between January 1, 2001 and June 30, 2009. The samples were retrospectively reviewed, and 18 samples were positive for C. perfringens. The medical records of these 18 patients were reviewed for age, gender, underlying disease, past illnesses, results of physical and laboratory testing, and radiographic data. Results All patients were diagnosed with SIRS. Fifteen patients (83.3%) were >65 years old mean age, 75±2 years (range, 59-88 years). There were more men (13) than women (5). The blood cultures were obtained from patients in various wards: tertiary care center (8), emergency room (5), surgical ward (4), and medical ward (1). Hepatobiliary tract diseases such as gallbladder stones and hepatic carcinoma were the most frequent underlying diseases (8). Five patients died, resulting in an overall mortality rate at 30 days of 27%. In the non-survival group, patients presented with septic shock (4) and gas-forming infection (2), and with significantly lower fibrinogen levels than those in the survival group. Septic shock at initial presentation was significantly associated with 30-day mortality for C. perfringens infection. Discussion and Conclusion There were no specific characteristics among clinical features of C. perfringens infection accompanied with SIRS. This may indicate that, in emergency rooms, diagnosing and initiating appropriate treatment for C. perfringens infection may be considerably difficult. It is important to be especially vigilant in identifying patients with C. perfringens infection underlying SIRS, and accompanied by shock.