BACKGROUND: Congenital dermal sinuses represent cutaneous depressions or tracts that are lined by stratified squamous epithelium. They communicate between the surface of the skin and deeper structures and may occur anywhere along the craniospinal axis. These sinuses are thought to result from abnormal separation of the cutaneous and neural ectoderm between the third and fifth week of intrauterine life. They may be often accompanied by other cutaneous stigmata, various dysraphic abnormalities, or intraspinal tumors. In the sacrococcygeal area, cutaneous congenital abnormalities are relatively common. It is estimated that 2% to 4% of children harbor intergluteal dorsal dermal sinuses. These intergluteal sinuses in the perianal region are frequently referred to as pits or dimples. Their cause is considered similar to other congenital dermal sinuses and appears unrelated to acquired pilonidal conditions observed in adults. They may become susceptible to local recurrent infection from trauma or hirsutism. Controversy regarding the evaluation and management of cutaneous defects in the coccygeal region exists. METHODS: Both a literature review and a career review of clinical material were performed. Databases for articles published in English were surveyed for key words relating to coccygeal sinuses using standard computerized search techniques. The medical records of children presenting to our neurosurgical clinic for evaluation of dorsal dermal sinuses were reviewed to identify those with intergluteal sinuses. RESULTS: In the evaluation of reported cases and of our own, we were unable to identify any children with coccygeal sinuses without other cutaneous markers other than hair with findings suggestive of intraspinal communication. CONCLUSIONS: Intergluteal dorsal dermal sinuses are relatively common lesions that frequently come to neurosurgical attention. They do not seem to be associated with significant risk of spinal cord and intraspinal anomalies. Simple intergluteal dorsal dermal sinuses without other cutaneous findings do not require radiographic or surgical evaluation and treatment. If other markers or neurologic symptoms are present, however, radiographic evaluation may be indicated.