Dysraphism refers to neural tube closure abnormalities and midline closure abnormalities of the skin, paravertebral muscles, vertebrae and meninges. Cranial dysraphism (CD) and occult spinal dysraphism (OSD) may be discovered via evocative skin signs present at birth or appearing later in childhood or even in adulthood. This review describes the various types of skin signs associated with CD and OSD. All congenital midline skin lesions, particularly on the frontonasal area, the vertex or the occipitocervical and low back regions, should prompt suspicion of underlying dysraphism. The main evocative midline skin abnormalities are: (i) for underlying DCEO: a nodule, swelling, skin openings and hair collar sign or hair tuft; (ii) for underlying DSO, localized hypertrichosis, an atypical or complex lower back dimple, a dermoid fistula, infantile haemangioma, caudal appendage and lipoma. In the event of suspected DCEO or DSO, spinal or medullary MRI constitutes the reference examination.

Caudal appendage is a rare malformation which has since ever been interesting. We present the case of a girl in which a caudal appendage was discovered before birth. A throughout checking found an extension from this abnormality to the cordal spine. The baby is operated when 2months old. Differential diagnosis are spina bifida, teratoma and pilonidal sinus. Caudal appendage is one of the typical cutaneous finding with underlying spinal dysraphism, such as hair tuft or pigmented macule. The risk is a tethered cord syndrome that can lead to severe complication. The treatment is either surgery or expectation.