Children with underlying medical and developmental conditions often present with complications that include feeding difficulties. Severe feeding difficulties may meet the criteria for the psychiatric diagnosis of Avoidant Restrictive Food Intake Disorder (ARFID). ARFID is a disruption in feeding/eating which may include weight loss/faltering growth, nutrient deficiencies, and/or reliance on a nutritionally complete formula to meet caloric needs. Children with interstitial lung disease (ILD), have a higher prevalence of feeding problems than the general population. To date, no published literature exists to provide guidance for treating severe feeding difficulties experienced by children with ILD. In this paper, we present an encounter in which we safely and effectively treated ARFID as evidenced by underweight and dependence on a nutritionally complete formula in a 3-year-old male with ILD using a multidisciplinary approach to treatment. Before the admission, the patient relied primarily on oral nutritional supplementation for most of his nutritional needs and therapeutic efforts to advance food intake were complicated by mealtime behavior problems and concerns regarding swallow safety. Intervention introduced 16 new foods, established consistent caloric intake of target food, and successfully weaned the patient from bottle feeding. We also review special considerations and provide guidelines for adapting treatment at multidisciplinary programs for patients with complex cardiorespiratory disease including modifications to behavior management procedures, medical monitoring, and nutritional consultation.