Cysts and fistulas located on the dorsum of the nose are rare occurrences. These malformations are strictly confined to the midline, extending from the columella to the glabella. In some cases, they may extend deeply, potentially reaching the leptomeningeal spaces. Imaging studies are crucial for determining the depth of extension and the relationship between the malformation and surrounding structures, such as the cribriform plate and meninges. Detection of frontal and/or nasal bone defects on computed tomography scans or the presence of a transosseous canal with or without a cyst on magnetic resonance imaging should raise suspicion of a dermal sinus cyst prompt surgical excision is indicated to mitigate the risk of the most concerning complication: Neuro-meningeal superinfection. Preferably, surgical excision should be carried out before the age of 1 year to minimize the risk of infection. If communication with meningeal spaces is suspected, neurosurgical intervention becomes necessary. Herein, we present the case of a 4-year-old male child with a history of neonatal distress, psychomotor delays, and epileptic seizures. The patient presented a fistula at the columella level, accompanied by pus discharge, within the context of dysmorphic syndrome. Consequently, the patient was scheduled for fistula excision.