Lipoid proteinosis is a rare autosomal recessive disease that has recently been shown to result from mutations in the ECM1 gene. Some cases are associated with a more severe mucocutaneous phenotype.We report the case of a 38-year-old woman who had clinical and histological skin features typical of Urbach-Wiethe disease. Physical examination revealed neurological abnormalities including dizziness, ataxia, slight psychomotor retardation and amnesic impairment. The patient reported sudden left brachiofacial paralysis one month earlier that regressed spontaneously after one week. CT scan and MRI were normal. Cerebral scintigraphy displayed bilateral hypoperfusion of the frontal areas, of the anterior and internal right temporal lobe (which includes amygdala), and of the left thalamic core.We considered these abnormalities as neurological features of lipoid proteinosis in the absence of evidence of any other cause. Our observation as well as other recent reports suggests the need for neurological and neuropsychological investigations in patients with Urbach-Wiethe disease.