Although some of the cases may present intranasally, most of them exhibit as external nasal masses with a tiny dimple along the midline of the nasal bridge visible at birth or in early childhood.

• 除了上述部位，偶而也可以在鼻窦、眼眶、颅腔及脑硬膜中枢神经等处发现。至于外鼻部的异常，则在出生或儿童时期，可以看到鼻梁中央有凹点的肿块。