Dysfunction of the urinary bladder and urethra due to diseases of the central nervous system or peripheral nerves is a major global medical and social problem. Nervous system abnormalities as well as traumatic spinal cord injury, spinal cord tumors, CNS infection can affect both adults and children. This chapter analyzes the most common neurological disorders in children, mainly myelodysplasia, which deals with neurogenic bladder dysfunction (NBD). The changing paradigm in the pediatric age is that there is a growing number of neurological diseases, both congenital and genetic, that deal with NBD. The steps of a diagnostic workup for those conditions starting at birth as well as the occult lesions detected later on are underlined. The main objective of modern management is the prevention of urinary tract deterioration by early detection of so-called “at-risk bladders.” A urodynamic study (UDS) is the most important diagnostic tool to detect, classify, and to follow-up the NBD. Reliable urodynamic investigations have changed the capability to evaluate the conditions at risk for upper urinary tract and renal function impairment significantly.