The purpose of this case is to demonstrate a spontaneous and topic pregnancy in a didelphic uterus, highlighting the importance of complementary evaluationof the urinary tract. Patient with 30 years, gravida 2, para 0, abortion 1, complaining of vaginal bleeding and pelvic pain for 3 days. The medical history of the patient only demonstrated a spontaneous miscarriage 2 years ago. The gynecological and obstetric examination was normal. The transvaginal ultrasonography demonstrated two separate uterus, two impervious cervices with no intravaginal septum and topical viable pregnancy in the right uterus with estimated date of 8 weeks. The evaluation of the left uterus demonstrated endometrial thickening compatible with secretory phase of the menstrual cycle. We extended our ultrasound exam into the abdominal cavity and diagnosed right renal agenesis with left vicarious kidney. After the first trimester, the urinary tract and the uterus imaging study was complemented with MRI. Didelphic uterus with pregnancy on the right side; right kidney agenesis. This case illustrates the importance of understanding the different types of uterine malformations and their possible comorbidities. The correct diagnosis is extremely important for the correct conduction of the prenatal and its complementary imaging study.