A rare case of atraumatic splenic rupture due to ceroid histiocytosis is described in a 56-year-old man. During hospitalization, he complained of epigastric pain, which was not associated with nausea or vomiting. Hematologic indices showed steadily declining hemoglobin, and subsequent radiologic imaging revealed massive splenomegaly. An exploratory laparotomy revealed hemoperitoneum associated with a subcapsular splenic rupture. Splenic pathology showed numerous ceroid-containing histiocytes. We believe this to be the fourth reported case in the English language of atraumatic splenic rupture due to ceroid histiocytosis.