Thoracoabdominal syndrome (THAS), also known as pentalogy of Cantrell (POC), is an extremely uncommon, fatal congenital disorder with an undetermined inheritance pattern (Williams et al. 2019; Fabijan et al. 2024). Although there were documented cases of THAS in the early 1700s, Cantrell et al. first characterized a syndrome distinguished by multiple anomalies comprised of distal sternal defect, ventral abdominal wall deformity in the midline, an anterolateral diaphragmatic defect associated with omphalocele, abnormality of the apical pericardium, and intracardiac malformations (Cantrell et al. 1958). Toriello and Higgins studied five males who were connected through women and had six varying anomalies that appeared in either the midline or as midline-associated abnormalities, including cleft lip, hydrocephalus, anencephaly, congenital heart anomalies, renal agenesis, and hypospadias (Toriello and Higgins 1985). As formerly stated by Opitz and Gilbert, Toriello and Higgins assumed that the midline may be a developmental field and a single gene mutation, in this case on chromosome X, resulted in defective development (Opitz and Gilbert 1982; Toriello and Higgins 1985). However, Carmi and Boughman determined five cases of THAS, including at least two females (Carmi and Boughman 1992). The prevalence of THAS is estimated to be between 1:65,000 and 1:200,000 cases, with a male predominance of 1.35:1 (Ergenoğlu et al. 2012; Mărginean et al. 2018). THAS incident rate is found to be 5.5 to 7.9 per million live births (Carmi and Boughman 1992). Several THAS subjects had been accompanied by chromosomal defects, such as trisomy 13, trisomy 18, trisomy 21, or Turner’s syndrome (Williams et al. 2019). Thoracoabdominal wall anomalies can result in omphalocele and ectopia cordis (EC), where the heart is positioned outside the thoracic cavity, occasionally covered by thin, tinted skin (Sana and Rentea 2024). Affected neonates typically exhibit dramatic respiratory distress, cyanosis, and severe respiratory infection secondary to pulmonary hypoplasia (Carmi et al. 1990; Chandran and Ari 2013; Williams et al. 2019). The accumulation of cardiac defect, cleft sternum, and ventral abdominal wall anomaly forms a pulsatile swelling under the skin visible at birth. THAS manifests during prenatal and antenatal periods (Engum 2008).