Background: Binder's syndrome or maxillonasal dysplasia is a rare developmental anomaly affecting the anterior maxillary region and nasal complex. Its causes are unclear and it is characterized by an extremely flat nose and retruded midface with a concave profile. Case description: We describe here a case of an 18-year-old male patient who presented with external nasal deformity since birth. On examination, the patient had classical features of the condition described by von Binder in 1962. Using costal cartilage graft, the nasal dorsum and the premaxilla were augmented by combined oral vestibular and external rhinoplasty approach. A brief review of the anomaly and the proposed treatment options in accordance with the age and the degree of malformation has been discussed. Conclusion: Satisfactory correction of the maxillonasal deformity, to achieve pleasing esthetics without functional limitations, is a challenge for surgeons. Clinical significance: This case report highlights the importance of correctly diagnosing patients suffering from Binder's syndrome (maxillonasal hypoplasia) and providing them with appropriate management. If clinicians miss this diagnosis, the desired surgical outcomes may not be achieved by rhinoplasty only without premaxillary augmentation.