Congenital hypertrichosis universalis is a rare autosomal dominant disease. We 
report the further development of a Greek girl, now aged 3 years, the first case 
associated with a balanced structural chromosomal aberration. She was described 
as a neonate by Sigalas et al. (1990). Her persistent generalized hypertrichosis 
is most excessive on the face, ears and shoulders. Her fine silky hair is of the 
vellus, not the lanugo type. The syndrome features are characterized, referring 
to nine further published case reports. It is distinguished from other types of 
congenital hypertrichoses, which have been described in the literature under 
different synonyms. To avoid confusion in the terminology, we propose to name 
this type of hypertrichosis Ambras syndrome in reference to the first documented 
family with congenital hypertrichosis universalis in the 16th century.