Congenital chylous ascites (CA) is the rare accumulation of chyle in the peritoneal cavity, often occurring in infants under three months of age. In this case study, we report a case of congenital CA detected prenatally on routine 34-week fetal ultrasound in the presence of congenital hypothyroidism. Treatment consisting of thyroid hormone supplementation, a diet high in middle-chain triglycerides and octreotide administration was undertaken. To our knowledge, this is the second case of CA in the presence of congenital hypothyroidism described in the literature.