Congenital lobar emphysema (CLE) is a rare lung anomaly characterized by hyperinflation of one or more pulmonary lobes. It most often presents within the first six monthsof- life with respiratory failure. Treatment depends on disease severity, with resection of the affected lobe(s) necessary for children with severe respiratory compromise. Children with resected CLE may be followed by their surgeon, but more commonly, the long-term care of these patients falls to primary care providers, including physician assistants. As CLE is rare, primary care providers may not have the experience to assess and treat long-term outcomes as these patients grow up. The purpose of this study was to summarize the existing literature about the long-term outcomes of children with resected CLE and create a best practice guideline for the follow-up of patients after CLE resection. A literature search was performed, which revealed the 31 eligible references included in this review. The outcomes described in the references were grouped into five themes: survival, respiratory, musculoskeletal, anthropometric, and general well-being. Overall, the literature suggested that these children do well in terms of survival, growth and development, and general well-being. However, there are areas that primary care providers should screen for complications. Musculoskeletal complications such as pectus excavatum and scoliosis are more prevalent in children with resected CLE than in the general population. Additionally, children with resected CLE may be at increased risk of small airway obstruction and diminished, but not significant, pulmonary function.