Abstract: Congenital arhinia is a fetal malformation with unknown etiology, unclear pathogenesis and extremely rare clinically. It often accompanied by other organ malformations, which affects the outcome of perinatal infants in varying degrees. Therefore, a skilled neonatal resuscitation team is needed during delivery. Most fetuses with arhinia can live normally after birth. At present, surgery is still the main therapeutic method for arhinia, and stem cell therapy is expected to become a new treatment method. This paper reported a case of arhinia of fetus admitted to our hospital, and the relevant literature was reviewed. The purpose of this study is to raise awareness of the disease, reduce missed diagnosis and misdiagnosis, provide necessary prenatal counseling and help for patients and their families, and give sound advice for neonatal care, feeding and treatment.