Midline nasal congenital masses are a rare congenital anomaly. Most often such defects include: vascular tumors, benign and malignant tumors and inflammatory lesions, however the most common are: gliomas, dermoid cysts and hemangiomas. Dermoid cysts (or dermoids) are a benign tumor belongs to choristom group (teratoma). Nasal dermoid cysts (NDCs) are the most common congenital defect of the nasal midline. The diagnosis is usually established at an early age - mainly by the age of 3. Due to late diagnosis and/or delay surgical treatment, facial deformities, recurrent infections (festering of cysts or subcutaneous infections), nasal blockage or intracranial complications may occur. This paper presents a clinical case of a 7-year old boy with a nasal dermoid cyst, successfully treated surgically with open rhinoplasty and follow-up period of 2 year.