Introduction: Mycoplasma pneumoniae is an important causative agent of respiratory infection in children. M. pneumoniae has been known to cause various extrapulmonary diseases in almost every organ. We describe a case of septic shock related to M. pneumoniae infection in a child without respiratory manifestations. Case Report: An 11-year old girl was admitted due to fever for 3 days and generalized weakness. She had developed erythematous macular skin rash on whole body and both eye hyperemia the day before the admission. She had no respiratory symptoms and initial chest radiograph was normal. However, initial laboratory tests showed azotemia and marked elevation of C-reactive protein. On the next day her renal function deteriorated rapidly, and oliguria and hypotension were noted. The patient was transferred to the intensive care unit. She was able to recovery by aggressive supportive care. M. pneumoniae infection was diagnosed by a 4-fold increase in antibodies to M. pneumoniae between acute and convalescent sera. No other etiologic factors or pathogens were detected. Conclusion: M. pneumoniae can cause severe atypical infection without pulmonary symptoms. M. pneumoniae infection should be considered a cause of children with sepsis or unusual presentation of severe infection.