We report a 52-year-old female patient admitted with fever, chills, and myalgias since the previous day. On the day of admission she had a generalized seizure. The patient had no previous illnesses. Laboratory investigations showed consumptive coagulopathy with clinical manifestations of shock and development of multiple organ failure. Pneumococci were detected in blood cultures. Furthermore the skin showed purpura fulminans all over. The patient died within 24 h after admission in the intensive care unit. On autopsy, in addition to adrenal and myocardial hemorrhages, hypoplasia of the spleen was found. Fulminant pneumococcal sepsis is a life-threatening disease that occurs in patients with risk factors like splenic hypoplasia or asplenia. Sometimes a fulminant pneumococcal sepsis may be the first clinical manifestation of a hitherto unknown splenic hypoplasia. In this context the general recommendation of vaccination against pneumococci in patients with risk factors like splenic hypoplasia or asplenia, in patients older than 60, and in children from 2 months onward has to be emphasized.