Epignathus or teratoma arising from the hard palate nasal cavity is an extremely rare abnormality. This tumour usually causes death in neonatal life because of its location and because surgical removal is often impossible. We are presenting a case of epignathus originating from the ethmoidal sinus delivered at gestational week 29 by caesarean section due to increasing hydramniosis. The child died within a few minutes. Prenatal diagnosis is important for genetic counselling, obstetric management and, in some cases, neonatal surgery.