Abstract: Cloacal exstrophy in its most complex form with OEIS syndrome is characterized by the existence of an Omphalocele, Exstrophied bladder, Imperforate anus and Spina bifida. We report a particular case of Cloacal exstrophy with OEIS complex and further birth defects. The diagnostic andtherapeutic approaches, as well as difficulties encountered in a limited resource setting are highlighted. This was a 1-day old neonate referred for the management of multiple congenital malformations, including antenatal diagnosis of malformative uropathy. On admission, the clinical findings included: a type 1 omphalocele, an anorectal malformation with a recto-urinary fistula and a covered lumbosacral dysraphism. Paraclinical examinations with cardiac and transfontanelle ultrasound revealed associated cardiac anomaly and findings in favor of lombo-sacral dysraphism. Supportive care was given and surgical reconstruction of birth defects on day 20 was done. In conclusion, cloacal exstrophy is a rare morbid congenital polymalformative syndrome in neonates, especially when presenting with OEIS complex or syndrome. They require prompt diagnosis and immediate postnatal multidisciplinary management, with long-term follow-up for a favorable outcome.