Imaging features are impressive of Mayer-Rokitansky-Küster-Hauser syndrome type 1. MRI, in this case, clarifies that agenesis of the uterus with renal anomalies is the atypical and rare form of MRKH syndrome and differentiates from other differentials including MRKH type 2 and hematocolpometra due to an imperforate hymen.