Multicystic dysplastic kidney (MCDK) is a common congenital condition in neonates caused by the abnormal formation of renal tissue into multiple fluid-filled cysts that are often dysplastic, affecting kidney function. [1] It has an incidence of 1:4300 live births and is more common in males than females, most often presenting unilaterally with a slightly higher percentage of cases affecting the left kidney. We present a case of a monochorionic diamniotic twin female, with twin A having multicystic dysplastic kidneys on the left and twin B having agenesis of the right kidney. Documented cases involving twins rarely involve both twins or, if so, infrequently in the contralateral kidneys of each twin, highlighting the complexity of renal development in utero.