A term neonate (TN) of 2 days, delivered by lower segment caesarean section in a private nursing home of a rural area, was referred to the outpatient department of paediatric surgery with a chief concern of large abdominal swelling and mild respiratory distress. The TN was diagnosed with giant omphalocele and a right diaphragmatic hernia.Intraoperatively, the omphalocele sac was found to contain liver, stomach, spleen and small intestine, with the latter herniating through a right diaphragmatic defect. Additionally, the lower part of the heart was adhered to the under-surface of the sac in the right upper hypochondrium. The coexistence of a giant omphalocele, right diaphragmatic hernia and partial Cantrell's pentalogy (POC) makes this case unique, highlighting the need to consider this anomaly in similar presentations.