本文报道了在同胞姊妹中同时出现两位不完全性男性假两性畸形Ⅱ型的病例,两患者社会性别均为女性,但患者具有睾丸,无子宫及阴道,外周血细胞染色体核型分析为46xy、口腔粘膜细胞X染色质阴性,患者祖母与外祖母系亲姊妹,父母为近亲结合,亲缘系数r=1/8,均为杂合子,其子女8人中,有二位患者,为纯合型,占1/4,符合常染色体隐性遗传方式。 In this paper, we report two cases of incomplete males and bisexual deformities in sibling siblings. The two sexes were female, but the patients had testicles, no uterus and vagina. The karyotype analysis of peripheral blood cells was 46xy, Oral mucosal cells X-negative, the patient’s grandmother and his grandmother’s sister, parents of close relatives, genetic factors r = 1/8, are heterozygous, 8 of their children, two patients were homozygous, Accounting for 1/4, in line with autosomal recessive inheritance.