Sirenomelia is a rare anomaly of the caudal region of the body, presenting with fusion of the lower limbs. Genitourinary, gastrointestinal, neural tube and vertebral anomalies are found in most cases. Herein, a very rare case with the sirenomelia sequence is presented. The patient had fusion of both lower limbs, absent genitalia, absent anal orifice, absent ureteral orifice, pelvic and sacral dysplasia and a single umbilical artery. In addition, he had a malpositioned penis in the posterior sacral area. He died 11 hours after birth.

Keywords: Malposition of the penis, Oligohydramnios, Sirenomelia