Abstract: Marie Unna hypotrichosis is a rare, autosomal dominant hypotrichosis characterized by sparse or absent hair at birth with regrowth of coarse, wiry hair from childhood, followed by progressive loss on approaching puberty. We report a Chinese mother and child seen at our skin center with features of Marie Unna hypotrichosis. The family pedigree showed two affected sisters and one affected brother, consistent with an autosomal dominant mode of inheritance. The clinical, genetic, histologic, and ultrastructural features were consistent with the diagnosis of Marie Unna hypotrichosis. This is the first Chinese family reported.