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We report a case of human herpesvirus 6 (HHV-6) encephalomyelitis in an immunocompetent patient, which was confirmed by viral amplification from cerebrospinal fluid. Cidofovir was used, followed by ganciclovir, because of an adverse effect to probenecid. The patient recovered. HHV-6 should be recognized as one of the causes of encephalomyelitis.