G.D., a 79 year-old female, presents with a severe and slowly progressive amnesia although she remains entirely independent in daily life and is perfectly well spatially oriented. Her amnesia is relatively isolated and her deficit does not embrace other cognitive domains. G.D. underwent extensive neuropsychological evaluation including language, executive functions, perceptual, and memory tests. Based on clinical observation, the purpose of this study was to determine whether there was a dissociation between her autobiographical and semantic memory. Results point out a severely degraded semantic knowledge of famous public events and persons while autobiographical memory of personally experienced and relevant information remains intact. Results from this study and from previous studies seem to suggest that relative sparing of hippocampal structures may be related to the preservation of autobiographical memory.