It is common for oral health and dental care to be considered a lesser priority for children with complex medical histories than other aspects of their health care. Often, these patients are at a high risk for caries and infection due to poor oral health practices at home, special or restricted diets, and no early establishment of a dental home for routine dental care. Unfortunately, many of these patients present to their first dental visits with caries and require aggressive treatment, such as extractions instead of pulp therapy, or crowns instead of fillings, due to their high caries risk and the difficulty in safely managing them medically during treatment. A unique example of this occurred at the Children's Hospital of Michigan, where a patient with Townes-Brock syndrome (TBS) presented to the dental clinic with advanced caries. TBS is a rare autosomal dominant disorder characterized by major findings such as anomalies of the external ear, imperforate anus, renal malformations, and malformations of the hand. Like many medically complex cases, dental anomalies are not a direct consequence of TBS; however, due to the necessity of high calorie and high sugar feeding supplementation, many of these patients are at high risk for advanced dental caries. Due to this high caries risk, a more aggressive treatment plan is necessary to minimize the risk of recurrent decay and infection. It is critical to stress that even if the disease, syndrome, etc., of a patient does not have inherent dental consequences, it is imperative for regular dental care to be part of the comprehensive treatment plan for these patients. This includes the establishment of a dental home at a young age and proper oral health education of the patient's caregivers and their physicians. In the case of the patient with TBS, recommendations for daily brushing, especially after high sugar feedings was stressed, as well as the reduction of any other sweets within the diet.