Creutzfeldt-Jacob disease (CJD) broadly involves the central nervous system and exhibits a wide variety of symptoms; consequently, patients are frequently misdiagnosed in its early stages with other neuropsychiatric syndromes. In the present paper, the authors report a case of CJD in a patient with alcohol dependence. A 65-year-old woman drank heavily for several years and occasionally had delusions of persecution. After a year of abstinence, she resumed drinking and exhibited behavioral anomalies. She was admitted to an internal medicine ward of a hospital because of an asthmatic attack. She then became unresponsive, bedridden and incontinent, and was moved to the psychotic ward of our hospital. From findings of rapidly progressing dementia, myoclonus, characteristic electroencephalogram, and signal increase in the cortex and basal ganglia on diffusion-weighted magnetic resonance imaging the woman was diagnosed with CJD. CJD is a rare but considerable syndrome in the differential diagnosis of alcohol-related disorders.