Francois综合征又名Ullrch-Fremerey-Dohna氏综合征或Hallermann-Streiff氏综合征;鸟头样自内障综合征或下颌-眼-面-颅骨发育不全综合征。临床上甚少见,其特征为颅骨发育异常、双眼小眼球、双先天性白内障、鸟头样、无毛发。我国除张方华报告18例。杨国华报告5例外,其它地区未见报道,我院收治二例,特报道如下。 Francois syndrome is also known as Ullrch-Fremerey-Dohna’s syndrome or Hallermann-Strehne’s syndrome; bird’s head-like cataract syndrome or mandibular-ocular-facial-skull dysplasia syndrome. Clinically rare, characterized by skull dysplasia, binocular small eyeball, double congenital cataract, bird-like, no hair. In addition to Zhang Fanghua reported 18 cases in our country. Yang Guohua reported five exceptions, no other reports, two cases admitted to our hospital, especially reported as follows.