Background: Pentalogy of Cantrell (POC) is an extremely rare and usually lethal congenital anomaly; to the best of our knowledge, less than 10 cases of first trimester diagnosis have been reported by using only transabdominal 2-dimensional (2D) ultrasonography.
Case Report: A 37-years-old Thai woman, gravida 3, para 2, 13 weeks of gestation presented at early pregnancy clinic for dating confirmation. Ultrasonography revealed a fetus with multiple anomalies, so she was referred to Maternal Fetal Medicine (MFM) unit. Transabdominal 2D ultrasonography demonstrated a single alive fetus with ectopia cordis and evisceration of liver and bowel loops. Then, the diagnosis of pentalogy of Cantrell was made. After counseling about poor prognosis of POC, the patient and her husband decided termination of pregnancy. The autopsy revealed scoliosis, herniation of chest and abdominal viscera organs including heart, lungs, liver, spleen, small and large intestines through a paramedian abdominal wall fusion defect represented the diagnosed POC.
Conclusion: The authors represent the first case of POC in Thailand diagnosed by transabdominal 2D ultrasonography in first trimester. Therefore, careful examination ultrasonography possibly provide an excellent view diagnosed lethal congenital anomaly in the first trimester which reduce mortality and morbidity to the patient with late termination.

Keywords: Pentalogy of Cantrell, Ectopia cordis, Evisceration of bowel loops