BRAF and familial atrioventricular septal defect: While in an adult, a nodular parenchymal BRAF V600E CNS-JXG diagnosed neoplasm with background CNS white matter changes and a cutaneous BRAF mutated xanthogranuloma lesion is highly suggestive of ECD [23], in children this presentation is not as well recognized as a form of pediatric ECD, especially in the pre-BRAF era [7].