The following report of a mother and two children with familial congenital defect of the scalp is presented because 1. It is an extremely rare condition. 2. It has never been reported before in the dermatologic literature. 3. The familial aspect helps evaluate previous theories as to the etiology of congenital defect of the scalp. Report of Case A mother and two children were seen because all three had an area of atrophic, cicatricial alopecia on the vertex of the scalp since birth. The mother stated that she was born with a "raw" area which healed, leaving a circular, depressed area of alopecia about the size of a quarter at the vertex. At the age of 8, for cosmetic reasons, a plastic repair was attempted and was partially successful. Both children, girls, aged 4 years and 11/2 years, were born with heart