We present three cases with atypical presentation of the Mayer-Rokitansky-Kuster-Hauser syndrome observed by US between August 1994 and December 1997. In two cases we observed complete absence of the uterine. In the last case, an uterus remnant was present. In all cases, both ovaries were normal and the three women reported "normal" sexual activity. An ectopic kidney was present in one patient with complete absence of the uterus. Atypical forms of Mayer-Rokitansky-Kuster-Hauser syndrome with normal vagina can be diagnosed at US. In the cases presented here, US allowed a clear description of the anatomy and provided a comprehensive evaluation of the patients.