In the last decade, the gain-of-function BRAFV600E mutation was observed to be recurrent in the histiocytic disorders Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD), characterized by the infiltration of CD1a+CD207+ and CD68+CD1a− clonal mononuclear phagocytes, respectively [1]. Here, CD207 is linked to familial atrioventricular septal defect.