Patent urachus cyst is a rare umbilical anomaly, which is poorly detected prenatally and frequently confounded with pseudo bladder exstrophy or omphalocele. A 27-year-old woman was referred to our prenatal diagnosis centre at 18 weeks of gestation after diagnosis of a megabladder and 2 umbilical cord cysts. Subsequent 2D, 3D and 4D ultrasound examinations and fetal magnetic resonance imaging (MRI) revealed a typical umbilical cyst and an extra-abdominal cyst, communicating with the vertex of the fetal bladder through a small channel that increased in size when the fetus voided urine. Termination of pregnancy occured at 31 weeks because of associated cerebral septal agenesis, and autopsy confirmed the prenatal diagnosis of urachus cyst. Few cases of urachus cyst diagnosed prenatally are reported in literature, but none were associated with other extra-abdominal disorders and none used 3D, 4D and fetal MRI. Our case illustrated the efficiency in prenatal diagnosis of 3D and 4D ultrasound examinations. This could help pediatrician surgeons to explain to a couple about neonatal surgical repair and plastic reconstruction in the prenatal period.