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In symptomatic West syndrome, multiple brain pathologies have been demonstrated as causal factors. Usually the epileptic spasms are intractable and the patients remain in a state of severe intellectual disability. Because adrenocorticotropic hormone (ACTH) is a standard and the most effective therapy for intractable spasms, neuro-immuno-modulatory aspects have been postulated to be involved in the pathogenesis, but details remain unclear. We here present the clinical course of a girl with West syndrome due to neonatal intracerebral hemorrhages secondary to protein C deficiency whose intractable spasms and electroencephalogram (EEG) abnormality improved dramatically after liver transplantation which included tacrolimus administration. Recently, many studies have shown that activation of calcineurin is involved in the enhancement of cortical excitability through the control of GABAergic inhibition. We speculate that tacrolimus, as a calcineurin inhibitor, may have played a role in the improvement of her spasms following liver transplantation by promoting GABAergic inhibition. This is the first report that West syndrome can be improved by tacrolimus.