Aim: To present a rare case of nasal dermoid cyst and its management in a 14-year-old boy. Background: Congenital midline nasal masses are rare, with an incidence estimated to be 1 in 20,000 to 1 in 40,000. Among these, nasal dermoid cyst is commonest. They arise from the lines of embryonic fusion. Various theories have been postulated in the development of nasal dermoid cyst, and prenasal theory by Pratt is the most accepted one. Case report: A 14-year-old boy presented with a gradually progressive midline swelling since birth, which was solitary, soft, nontransilluminant, present over the lower dorsum and tip region. Based on the history, clinical examination, and serial relevant investigations, diagnosis of a nasal dermoid cyst was made. Surgical strategy planned was dermoid cyst extirpation via an open rhinoplasty approach and augmentation of the defect with an autologous conchal cartilage graft. Conclusion: Nasal dermoid cyst is a rare congenital anomaly. Presence of intracranial extensions, sinus formation, and other differential diagnosis should be ruled out preoperatively. It demands early surgical intervention in order to avoid further distortion of the nose or atrophy of the bony or cartilaginous component as a result of pressure secondary to the growth of cyst and infection. Clinical significance: • This midline congenital cyst is cosmetically unacceptable. Hence, early surgical removal is mandatory. • Open rhinoplasty is the preferred surgery as it gives good esthetic results. It gives a scope for augmentation of the defect following the extirpation of the cyst. • Autologous conchal cartilage is the preferred graft as it provides sufficient quantity and esthetically pleasing contour.