A 38-year-old man receiving cyclosporine A after bilateral lung transplantation for cystic fibrosis presented with cortical blindness, generalized seizures, and cerebellar edema. Progressive brainstem compression necessitated emergency posterior fossa decompression. Replacement of cyclosporine A with an alternative immunosuppressive agent, FK506, was followed by rapid neurological recovery and dramatic resolution of radiographic abnormalities. The etiology, clinical features, and radiographic findings of cyclosporine A neurotoxicity are discussed. The pertinent literature is reviewed.