A 68-year-old woman with schizophrenia after a cerebrovascular accident resulting in right medial temporal and occipital damage developed Frégoli syndrome. Neuropsychological testing revealed that she had impairment in facial recognition compatible with prosopagnosia. The Frégoli syndrome disappeared in three month when, remarkably, her prosopagnosia also disappeared. Our findings are consistent with the hypothesis that combination of hyperactivations of temporo-limbic connection and frontal dysfunction may account for the development of Frégoli syndrome.