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Segmental spinal dysgenesis is a rare and challenging entity especially when associated with occult dysraphism. Experience with a female patient followed during a period of 10 years spanning from 5 to 15 years of age is reported. During that period the girl underwent three spinal operations consisting in one decompression and spinal cord untethering, one posterior instrumented fusion and a spinal cord re-untethering. Clinical and radiological features are discussed and considerations on optimization of management strategies are made.