BRAF and familial atrioventricular septal defect: While our CNS-JXG pediatric patient, with an associated BRAF V600E skin lesion did not have classic radiographic stigmata of ECD and has thus far responded to clofarabine and dexamethasone with clinical and radiographic improvement, the background radiographic features suggestive of ECD-related neurodegeneration should be further followed in this setting.