Spinal dysraphism is one of the most frequent malformations, and is defined as a spectrum of congenital anomalies characterized by an incomplete fusion of the midline mesenchymal, bony, or neural elements of the spine.Both the skin and nervous tissue anomalies which originate from ectoderm may occur simultaneously. It can be occult or cystic. Occult forms (occult spinal dysraphism) are characterized by skin-covered lesions without exposed neural tissue. The diagnosis of the occult spinal disraphism is usually made based on a suspicion of the midline paraspinal skin lesions, mostly localized in the lumbosacral area such as subcutaneous lipomas, dermal sinuses, tails, sacral dimple, localized hypertrichosis, hyperpigmented lesions, hemangioma, mongolian spot, pigmentary nevus, and aplasia cutis congenita. Here we described four male babies with occult spinal dysraphism associated with unusual alopecic scalp lesions.