Because splenomegaly may result from NPC1 and the patient is treated NPC1 by Miglustat, an iminosugar inhibitor of glucosylceramide synthase, which approved in Europe and in a number of other countries for the treatment of neurological manifestations of NPC, BCG-itis was our first diagnosis and the immunomodulator was our preferred treatment rather than anti-tuberculosis. Here, NPC1 is linked to Splenomegaly.