Objective:To explore the clinical features of Heidenhain variant Creutzfeldt-Jakob disease (HvCJD) to deepen understanding and recognition of this disease.Methods:Clinical data of 1 case of HvCJD admitted to Hainan General Hospital on August 4, 2022, were collected, whose clinical characteristics were reviewed and analyzed, and literature review was conducted.Results:The 59 years old male patient initially experienced symptoms of blurred vision, followed by a rapid decline in cognitive function. Mini-Mental State Examination score was 21/30 and Montreal Cognitive Assessment score was 11/30. Diffusion-weighted magnetic resonance imaging demonstrated the presence of mild ribbon-like hyperintensity within the bilateral frontal-parietal-occipital-insular cortex. T 2WI fluid attenuated inversion recovery imaging exhibited slight hyperintensity within the bilateral parietal cortex. The electroencephalography showed atypical triphasic waves. The examination of cerebrospinal fluid demonstrated 14-3-3 protein with a positive result. Two months after onset of illness, follow-up revealed new symptoms of myoclonus in the patient. Finally, the patient was not effectively treated and died about 2.5 months after onset. Thirty-six relevant literatures of HvCJD were reviewed. Most Heidenhain variant patients exhibited occipital cortical diffusion weighted imaging hyperintensity. Posterior cortical damage may affect primary and higher-level visual processing, leading to various visual disturbances. The early symptoms were mainly visual symptoms, such as visual reduction, blurred vision, visual field defect and color vision impairment. Conclusions:HvCJD patients only present with various visual disorders at the onset, followed by other neurological symptoms. The disease progresses rapidly, and patients often die in a short period of time. The disease is very rare and is often misdiagnosed. Currently, there is no feasible and effective treatment for HvCJD.