[Moya-moya disease. Raynaud's phenomenon. IgA deficiency]

The authors report one case of Moya-Moya disease in a 3 month-old infant, who developed microcephaly, mental retardation and tetraparesis. The appearance in the following months of a Raynaud's phenomenon in the upper limbs and of IgA deficiency may be of interest for the understanding of the disease.