Patient is a 14-year-old female with Niemann Pick disease type C manifested primarily with seizures. This subcategory is a result of defective intracellular processing and transporting of low-density lipoprotein-derived cholesterol. She presented with acute abdominal pain and underwent abdominal exploration with splenectomy for splenic torsion. The spleen showed ischemic changes and was notable for a marked histiocytic proliferation, as illustrated in this image.