ABSTRACT Renal agenesis is the absence of renal tissue due to defects in early embryonic development. It is often associated with other congenital abnormalities. The incidence of bilateral renal agenesis ranges from 0.1 to 0.3 per 1,000 births, making it a rare condition. Its diagnosis is mainly based on obstetric ultrasound by direct and indirect signs. Magnetic Resonance Imaging (MRI) has a higher sensitivity and specificity, and has its place when in doubt or when other anomalies are suspected. Management of bilateral renal agenesis relies primarily on prevention of pulmonary hypoplasia during pregnancy by amnioinfusion and correction of postpartum complications related to renal absence. Although the prognosis for bilateral renal agenesis is extremely poor and the risk of death in the neonatal period is high. We report a pregnancy in which prenatal ultrasound revealed anhydramnios and the absence of both fetal kidneys, To overcome the difficulties in obtaining accurate information on fetal ultrasound in cases of oligohydramnios, we used fetal magnetic resonance imaging (MRI) to confirm renal status, which confirmed fetal renal agenesis and provided a more accurate diagnosis. The neonate was born with a complex dysmorphic syndrome and died 45 minutes after birth due to respiratory distress.