Abstract
We present 2 patients with multifocal, small, round to oval, red and pink spots, some with a white halo. The diagnosis of capillary malformation - arteriovenous malformation syndrome (CM-AVM) was made based on their (common) characteristic clinical features and confirmed genetically by documenting a (different) germline heterozygous mutation in the RASA1 gene. In patients with a genetically confirmed diagnosis of CM-AVM, we recommend that at least one brain MRI angiography be performed at baseline and on a low-threshold basis in individuals with symptoms to rule out intracranial arteriovenous malformations and arteriovenous fistulas.