Static encephalopathy of childhood with neurodegeneration in adulthood/beta-propeller protein-associated neurodegeneration (SENDA/BPAN) is a rare congenital genetic disorder. The clinical course for this disorder is very specific over time as patients progress from childhood to adulthood. Dysphagia, epilepsy, and intellectual disability were the main complicating factors in this case; however, the patient was planned to undergo intravenous (IV) moderate sedation for her dental care. Midazolam was selected as the first choice to establish a baseline level of sedation due to her epilepsy and intellectual disability. For dysphagia and to minimize excessive movement perioperatively, small 10-mg boluses of propofol were administered to prevent loss of the swallowing reflex, and the depth of sedation was maintained at a Modified Observer’s Assessment of Alertness/Sedation score of approximately 4, which was consistent with moderate sedation. Intraoral water use was kept to a minimum, and any injected water was rapidly suctioned away. We performed IV moderate sedation on a patient with SENDA/BPAN undergoing dental treatment and were able to complete the planned procedure safely using carefully titrated midazolam and propofol.