We report on two patients from one family with Marie Unna hypotrichosis who showed a diffuse hair defect which typically occurs as an isolated phenomenon. The ultrastructural hair findings consisted of longitudinal grooving of the hair shafts and a flattening in cross-section. Characteristically, scalp hair had been lost from the scalp margins and a high frontal and nuchal hairline had been present at birth. Eyelashes were absent, and eyebrows, axillary, and pubic hair were very sparse. Other affected cases have been reported in Caucasian families; however, this is the first case in the English medical literature of a family from Asia affected by Marie Unna hypotrichosis.