Uterus didelphys is a congenital anomaly of the female reproductive tract which arises from the abnormal fusion of the Mullerian ducts. We present, the first case to the best of our knowledge, of uterus didelphys with a unicavitary twin gestation to be documented in Ghana, a low-middle income country. A 24-year-old woman, gravida 3, para 0+2 miscarriages, was seen and admitted to our maternity ward due to elevated blood pressure with ++ proteinuria at 36 weeks of gestation. She attended an antenatal clinic regularly during the pregnancy but was mainly seen by midwives. Apart from multiple pregnancy, two 2D ultrasound examinations (one at 25 weeks gestation and another during admission) did not reveal any uterine malformations. At 37 weeks+2 days, she underwent emergency cesarean section on account of pre-eclampsia and a twin pregnancy with the leading twin in breech presentation. After delivering both babies and the placenta, the uterus was exteriorized and inspected, during which a non-gravid bulky left uterus was first found. Each uterus had a normal ovary and fallopian tube on its lateral end. Further postoperative examination revealed a normal-looking vulva, two vaginas, and two cervices. Both babies weighed 1.9 kg, each below the fifth percentile of weight for age. The elevated blood settled postoperatively and the postoperative period was uneventful. The patient and twins were found in a stable condition on review two weeks after delivery and the twins were healthy at 5 years. Despite being a rare presentation, we wish to create awareness among health workers in rural and low-resource settings of such cases and highlight the need to improve prenatal diagnostic capabilities, as this is key to determining the mode of delivery and achieving favorable maternal and fetal outcomes.