Aim and objective: The aim of the article was to throw light on the potential risks associated with solitary kidney, especially in relation to maternal and fetal outcomes. Background: Solitary kidney can be caused by three main reasons: Being born with a single kidney, losing one because of a disease, or an injury, or donating a kidney to a family member or friend who has lost one of their own. Case description: A 38-year-old G7P4A2 patient with a 36.2-week period of gestation was admitted with antepartum hemorrhage. The patient was thoroughly examined and necessary investigations were done. On pelvic examination, there was mild spotting present. On routine investigations, she was found to be severely anemic and chronic kidney disease stage 5. According to the nephrology team's advice, an ultrasound of kidney, ureter, and urinary bladder (KUB) was done. It was detected as a case of congenital unilateral agenesis of the kidney. She had complaints of intermittent mild loin pain and dysuria. The patient was immediately transfused four units of packed cells for correction of anemia and was given steroid cover for fetal lung maturity. The patient was taken for hemodialysis in view of end-stage renal disease. After one session of hemodialysis, the patient was taken up for Cesarean section in view of placenta previa with a deranged renal profile. A single alive female baby weighing 2200 g was extracted with an Apgar score of 8, 9. The intraoperative period was uneventful. The patient was transferred to the intensive care unit for postoperative care. Conclusion: Solitary kidney from renal agenesis is a significant and independent risk factor for adverse maternal and fetal outcomes. Clinical significance: As unilateral renal agenesis (URA) is associated with potential risks, hence preconception counseling, close monitoring throughout their antenatal and postpartum period, and a multidisciplinary approach are required to have good pregnancy outcomes in these patients.