Preexisting neurological disease in pediatric patients presents unique challenges to the anesthesiologist. In‐depth knowledge of the disease processes and awareness of sequalae that uniquely influence the risks and benefits of anesthetics are needed to make informed decisions. Because these vulnerable populations are often susceptible to perioperative airway or cardiopulmonary complications, the use of regional anesthesia can be advantageous. However, these clinical conditions already involve compromised neural tissue and, as such, create additional concern that regional anesthesia may result in new or worsened deficits. The following discussion is not intended to be a full review of each disease process, but rather provides a concise, yet thorough, discussion of the available literature on regional anesthesia in the more common, but still rare, pediatric neurological disorders. We aim to provide a framework for pediatric anesthesiologists to reengage in a healthy discussion about the risks and benefits of utilizing regional anesthesia in this vulnerable population.