Case Report: A 35-year-old lady, G5P3, had a routine fetal anatomy scan at 22 weeks gestation. There were cleft lip and palate, hypoplastic nasal bone, nuchal fold oedema, bilateral pleural effusion, bilaterally enlarged echogenic lungs with heart compression and severe ascities. It was discovered, that the couple has had a neonatal death during her first pregnancy. Her first pregnancy which was also diagnosed to be syndromic and anhydramnions at 19 weeks. Unfortunately there were no formal karyotyping or post mortem done.

For this current pregnancy, the couple had extensive counselling with the genetic team and feto-maternal consultants, amniocentesis was offered. However couple decline and refuse for post mortem of the baby. Patient was agreeable for termination of pregnancy at 25 weeks, she delivered a female fetus, 780 gram. The fetus died shortly after birth. A thorough examination of the baby was done. The baby was dysmorphic, cryptophthalmos, flat nasal bone, low set ears, cleft palate and lip, syndactyly of the left fingers (3/4/5^th fingers) right fingers (2/3/4^th fingers) and clitromegaly.

Discussion: Congenital high airway obstruction syndrome (CHAOS) is a very rare and sporadic condition. It happens when foetal airway is complete or partial obstructed. It can vary in severity and that determines the foetal survival outcome. Although in majority of cases the associated anomalies related with CHAOS are not compatible with life and will lead to either stillbirth or death shortly after birth. There is some association between CHAOS and FRASER syndrome which is a autosomal recessive inheritance.  Thus, with this case report it will highlight the potential connection of CHOAS and FRASER autosomal inheritance pattern as evidence by repeat congenital abnormality in the subsequent pregnancy. The exact incidence of CHAOS associated with Fraser syndrome is unknown due to the rarity of this condition. In this case, it highlights the importance of identifying the ultrasonography features and matching it with a potential genetic condition. Ideally a karyotyping of either the fetus or parents might help aid further diagnosis. However when faced with patients that refuse for further genetic testing. A multi-disciplinary team consisting of feto-maternal consultants, genetic team and paediatric team is warranted when facing with complex multiple fetal congenital anomaly that is only diagnosed by ultrasonography features without abnormal karyotype results available. With the right approach and counselling a rare and complex case such as this can be managed excellently.