Midline congenital fistulae of the nose requires early diagnosis to av
oid infectious complications. This dermoid cyst of the nose may have i
ntracranial extension and is the consequence of an anterior neural tub
e closure abnormalitiy. The authors report 2 cases revealed by ophthal
mic complications. Computed tomographic scan or magnetic resonance ima
ging provides precise information concerning the extent. Surgical trea
tment is always necessary.