‘‘CHARGE association’’is a well-known entity of unknown origin. It was originally delineated by Bryan Hall [1979] in 17 children with multiple congenital anomalies (MCA) including choanal atresia and, independently by Hittner et al.[1979] in 10 MCA patients with coloboma, hence the eponymic Hall–Hittner syndrome which is sometimes used for it Graham, 2001. Pagon et al.[1981] coined ‘‘CHARGE,’’an acronym summarizing the five cardinal clinical features: ocular Coloboma, Heart defects of any type, Atresia of the choanae, Retardation (of growth and/or of development), Genital anomalies, and Ear anomalies (abnormal pinnae or hearing loss). Classically, to make a diagnosis of CHARGE complex, 4/7 signs need to be present [Oley et al., 1988], and one should be either choanal atresia or a coloboma [Pagon et al., 1981]. Many other developmental anomalies have been reported in CHARGE beyond the acronymic components [Pagon et al., 1981; Oley et al., 1988; Blake et al., 1998; Tellier et al., 1998; Gorlin et al., 2001]. Among those, otic malformations, anomalies of the cranial and CNS midline structures, hypophyseal disorders, and brainstem dysfunctions have to be stressed as emerging problems hidden beyond the historical cardinal features.