We describe a case of complete nasal agenesis and absence of the nasal fossae, without alterations in the central nervous system. The physical and intellectual development of the infant to date has been absolutely normal. Opening of the nasal respiratory passage was not required in our patient as he did not show respiratory problems during deglutition. - Microphthalmia in the right eye with iridoretinal coloboma and right cryptorchidism were also noted. When the child was 9 months old a right orbital asymmetry became evident due to a growth deficit of the microphthalmic eye. This improved after placement of an expandable prosthesis in the orbit to stimulate its growth. - When the child is 4 years old, he will start to use a nasal prosthesis supported by implantology. Final reconstruction of the nasal pyramid will take place after he is 15 years of age.