The authors report a female case of systemic lupus erythematosus (SLE) that was diagnosed as Wilson's disease (WD) when neurological manifestations were evident three years after the first admission. The brain imaging studies showed bilateral symmetrical basal ganglia involvement, slit lamp examination revealed Kayser-Fleischer ring of the cornea, and 24-hour urinary copper and serum ceruloplasmin also confirmed the diagnosis. The patient also had hemolytic anemia and hypoparathyroidism, which are rare presenting features of WD. SLE may be associated with WD, and presence of neurological, behavioral, or liver function abnormalities should raise the suspicion, even without apparent features of WD.