Introduction: Heteropagus is a asymmetrical category ofconjoined twins beside the diplopagus (symmetrical category).Wepresentacaseof a parasiticheteropagusattachedatthe chest wall with a rare presentation of giant exomphalos on the autosite and no intrathoracic organ involvement. Observation: A female newborn was referred to us at 7 days of age. The newborn was found to have a parasitic twin attached to the lower thoracic region, adherent to the sternum of the autosite twin. The birth weight was 3500 g including the parasite. The parasitic twin had two pelvic limbs and a rudimentary thoracic limb with no mobility connected by a pelvis with a perineum presenting female external genitalia but no anal orifice. Abdominal examination revealed a sessile omphalocele with an opaque membrane, measuring 9 cm. A thoraco-abdomino-pelvic CT scan revealed an anterior thoracic mass containing bony structures, suggesting limb segments one of which was in contact with the sternum. Echocardiography revealed multiple perimembranous and muscular ventricular septal defects. The diagnosis was an omphalopagus parasitic twin associated with an Aitken type 2 omphalocele. A conservative approach was adopted for the omphalocele, and surgery was performed at 20 days of life. At surgical exploration the parasitic pelvis, this contained a rudimentary kidney and a dilated ureter communicating with a distended bladder. The surgery involved excision of the pelvis and lower limbs, removal of the rudimentary upper limb, and resection of the osseous outgrowth. Daily dressings and antibiotic therapy for a suppuration observed led to favorable healing of the surgical wound. The outcome was favorable, with continued epithelialization of the omphalocele. Anaponeuroplastyis planned for 18 months. Conclusion: Adequate management of heteropagus conjoined twins and associated malformations require complete imaging evaluation.