Nasal dermoid sinus cysts (NDSCs) are rare neoplasms in the median line of the nasofrontal area. Unlike other dermoid cysts, a NDSC can manifest as a cyst, sinus, or fistula, and may extend intracranially. They usually occur as a mass in the median nasofrontal area and may have a pit. As an extensive tract can appear in some cases, careful preoperative evaluation and complete excision are required. A 15-year-old boy presented with a fistula, present from birth, in the nasal soft triangle. This report describes a case of NDSC occurring in the nasal septum and a fistula opening in the soft triangle, which is very rare, without recurrence after 4 years.
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