Providing anesthesia to patients with Brachmann-de Lange syndrome (BdLS) may be challenging, mainly because of intubation difficulty, gastroesophageal reflux disease, and aspiration complications. The use of spinal anesthesia (SA) in this population has not been reported. We report the uneventful administration of awake SA to a 7-month-old girl with BdLS who was scheduled for rectal biopsy. The current literature is reviewed to discuss the indications for SA in those patients.