Congenital lesions of the external nose appear during embryologic development. Their usual location is midline nasal area. The most common are dermoid cyst, glioma, encephalocele and meningocele. The histopatology confirms dermoid cyst in 60%. Diagnosis is based mainly on clinical examination of the patient. Computer tomography and magnetic resonance are useful to reveal the pathway of intracranial herniation or presence of associated anomalies of the cranial bones. A complete surgical removal of the lesion is the only effective treatment. In Plastic Surgery Clinic in Lodz, 20 patients 2-20 years old were operated between the years 1973-2004. Treatment consisted of simple surgical excision of the nasal lesion. The dermoid cyst was diagnosed in 55%. In 3 cases, where herniations were observed, a preparation and simple ligature was performed. The results of all operations were satisfactory. Congenital lesion of the external nose should be considered as dermoid cyst, glioma, encephalocele or meningocele. Complete excision is very important. Deformations of the nose after surgery are to be corrected after the facial skull growth process ends.