Congenital dermal sinuses are depressions or tracts lined by stratified squamous epithelium, which apparently result from defective separation of the neuroectoderm from the epithe-lial ectoderm along the mid-dorsal aspect of the embryo. If the sinus extends all the way into the spinal canal or cranial cavity, or if its walls are destroyed by infection, meningitis or abscess may result. The case is reported of a 5-year-old male who suffered two bouts of purulent meningitis of unknown etiology, associated with a congenital dermal sinus of the occipital region, with intracranial dermoid. X-rays revealed an underlying defect in the skull. He was treated surgically with excellent results. Congenital dermal sinuses should be looked for carefully in asymptomatic children as well as in cases of unexplained meningitis, particularly if due to staphylococci or of unknown etiology. The external appearance may be deceptively benign. The optimal time for excision of high lesions is before complications occur, and surgery should include preparations to enter the cranial vault if necessary. Patients with pilonidal sinuses require individual evaluation.