A rare case of isolated congenital bone agenesis in a young woman referred to the authors for aesthetic correction of the dorsum profile is described. There are no descriptions of surgical treatment for this isolated facial defect in the literature. After a complete and accurate diagnostic study, a rhinoplasty was performed with a good result and without complications during a 2-year follow-up period. To explain this congenital malformation, several hypotheses were examined, such as bony resorption and embryologic bony development syndromes. This report describes the possibility of intervention in the case of nasal bone absence and shows how risks of structural instability can be avoided with an accurate preoperative study and a careful surgical approach.