A midline dermal sinus tract is a manifestation of spinal dysraphism resulting from incomplete closure of the embryonic neuroectoderm early in development. The midline lesion with a central pit may be an important clue to the connection of the skin surface to the spinal canal, which occurs in approximately 50% of dermal sinuses.

Lesions occur most frequently in the lumbosacral region but can be seen anywhere along the midline (ie, cervical, thoracic, scalp regions) and in the genital area. Lateral congenital dermal sinuses may occur on the buttocks. They may be associated with other congenital skin lesions such as sacral hypertrichosis, lipoma, acrochordon / pseudotail, hemangioma, or capillary malformation. A multiplicity of congenital lesions over the midline raises risks for underlying spinal dysraphism.

Dermal sinus tracts can leak fluid (spinal fluid), and neurological deficits may be present. Meningeal infections, sometimes with an unusual organism(s), are a further complication.