Since Alport first reported hereditary nephritis with deafness in hematuria (also known as Alport syndrome, or AS) in 1927, observation and research on AS has been reported mostly in males with AS, and less in females. In reality, women with AS are not in the minority, as they are twice as likely as men in families with X-chromosome interlocking dominant AS (XLAS), which account for 85% of all AS families. The reasons for this are (1) the majority of female patients are from XLAS families (85% of all AS families), and their early clinical stage is usually mild and easily overlooked; (2) female AS patients have a greater chance of misdiagnosis and underdiagnosis than male patients; and (3) female patients have fewer opportunities to be seen, which does not exclude the influence of gender discrimination. In terms of the mode of inheritance, female AS also includes autosomal dominant Alport syndrome (ADAS), autosomal recessive Alport syndrome (ARAS) and X-chromosome interlocking dominant Alport syndrome (XLAS). XLAS in women is an X-chromosome interlocking dominant mode of inheritance, meaning that all women carrying the mutated gene express the abnormal gene product (heterozygotes for the gene also express the normal gene product), and XLAS families account for up to 85% of all AS families. The organs or sites include the glomerular basement membrane, the distal tubular basement membrane, the wall of Pau Mansfield’s bursa, the skin basement membrane, the crystal tegument (crystal tegument examination is less commonly performed clinically), and the inner ear vortex basement membrane. The next most common type of ARAS is COL4A3 or/and COL4A4, and the affected gene products are the a3 or/and a4 chains of type IV collagen molecules, and the organs or sites of expression include the glomerular basement membrane, proximal tubular basement membrane, crystal peritoneum, and inner ear vortex basement membrane; the relatively uncommon type is ADAS. The incidence of ARAS in women is the same as that of ARAS in men, and there is no clear difference in clinical manifestations and prognosis, whereas there are relatively few clinical reports of ADAS, and presumably no difference between the sexes.