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. The gene discussed is NPC1; the disease is tuberculosis.