We recently encountered a case of fatal pneumococcal infection in a previously healthy 19-year-old female. She had no history of splenectomy, but on autopsy she was found to have hyposplenism. It has been widely reported that life-threatening pneumococcal infection can occur after splenectomy, though cases of hyposplenic or asplenic adults, without a history of splenectomy, are very rare. We report this case and review the literature dealing with 6 similar cases.