Objective: To present a case of mullerian agenesis associated with agenesis of left kidney. Background: Advanced technologies used during the investigation helped a lot in understanding the developmental anomalies in female reproductive system as well as excretory system. With the help of modern technologies we often find the association of unusual presentation. Method: Here 16 year__ampersandsignrsquo;s old patient came with complains of not showing signs of menarche. She was examined and subjected to radiological investigations like ultrasonography (USG) Result: Abnormal structural presentation was noted. There was agenesis of uterus, cervix and vagina along with agenesis of left kidney. Conclusion: During the development there is a mutual and marginal differentiation exists between reproductive and excretory systems, are witnessed clinically with structural and functional abnormalities.