Tumors discovered antenatally are rare and the prognosis is generally poor. We present a case of a fetal left renal mass, initially discovered via routine prenatal ultrasound. The mother was an otherwise healthy gravida 4 para one 30-year-old female with 2 previous miscarriages, reportedly secondary to septate uterus. Further imaging and subsequent genetic testing was consistent with an antenatal Wilms’ tumor with a mutation in the NPHP1 gene. The newborn received chemotherapy and had no evidence of recurrence at 3 months follow-up.