The development of the kidneys and other organs of the urinary tract follows the natural rule of gene-environment-lifestyle interaction. Both intrinsic and extrinsic factors may be associated with the etiology of various kinds of urinary malformations, but the environmental factor is an extrinsic factor. Related literatures were reviewed in this paper, which focuses on the association of congenital urinary malformations with possible environmental factors. It is concluded that urinary malformation is associated with low birth weight, maternal disease, placental insufficiency, maternal drug exposure, and maternal exposure to environmental pesticides. Living environment and socioeconomic factors may also influence the incidence of urinary malformation.