Congenital atrichia may occur isolated or with associated defects. The isolated variant is especially uncommon. Sporadic and familial cases have been rarely reported. We present a large inbred kindred from Oman where 22 affected members with this disorder were found over six generations. The male-to-female ratio was 1:1.4. No associated ectodermal or other defects were noted. A typical autosomal recessive mode of inheritance was documented. Though inbreeding was high, anticipated quasidominance was observed only once.