A 32-year-old woman was diagnosed with leucopenia in 2002, being antinuclear antibody, anti-DNA antibody, and antiphospholipid antibody positive, and she was administered low-dose aspirin. In July 2006, she was admitted to our hospital because of pyrexia and abdominal pain. Examination revealed paralytic ileus, absence of the pupillary light reflex, dyshidrosis and anuresis. In addition, with high-level interleukin-6 in cerebrospinal fluid, the sensory nerve conduction velocity was derivation impotence. She was subsequently diagnosed with systemic lupus erythematosus with central nervous system involvement, peripheral neuropathy as well as acute pan-dysautonomia. After pulse corticosteroid therapy, paralytic ileus was improved, however, the urination disorder persisted, and syncope due to orthostatic hypotension became marked. Plasma exchange and a 2nd course of pulse corticosteroid therapy were performed, and were ineffective, whereas intravenous cyclophosphamide was effective. This patient is a rare case of central nervous system, peripheral neuropathy as well as acute pan-dysautonomia with systemic lupus erythematosus.