先天性孤立肾并肾盂输尿管畸形较少见,我们遇见一例,报道如下:患者女,10岁。自幼小便淋漓湿裤,增多次就医诊治,无效。查体:发育不良,身料矮小,智力正常,心肺无异常,腹软,肝脾未触及,尿道口有尿液溢出,余无特殊发现。静脉造影:右肾轮廓较低,下极达第四腰椎水平。于上、下极内各见一肾盂显示,但较正常为小,且肾盏数目较少,肾 Congenital isolated kidney and ureteropelvic deformity less common, we met a case, reported as follows: Female patient, 10 years old. Peeing dripping wet pants since childhood, increased medical treatment, invalid. Physical examination: poor growth, short stature, normal intelligence, no abnormal heart and lung, abdominal soft, liver and spleen not touched, urinary tract overflow urethra, I found no special. Venography: Lower right kidney contour, the fourth pole lumbar level. In the upper and lower pole see a renal pelvis, but smaller than normal, and less number of calyces, kidney