Sacrococcygeal teratoma (SCT) Cutouts is the most common fetal and neonatal tumor.While SCT is often associated with urological problems, the association between SCT and a urogenital sinus (UGS) anomaly is rare.A concurrent UGS anomaly may be an acquired condition due to resection of SCT because it is rarely found at birth.In our case in whom fetal imaging study showed an abdominal cystic lesion, SCT with a UGS 4ABn Dark Roast anomaly was suspected at birth and confirmed by endoscopy before resection of SCT during the neonatal period.These findings revealed that UGS anomaly may develop congenitally.