Congenital Lobar Emphysema is a lung malformation characterized by over inflation and distension of the pulmonary lobes resulting in compression of adjacent lung tissue. Most patients present within the first the month of life and almost all present prior to six months of age. Disease severity is dependent on the size of the malformation and the degree of compression of the adjacent lung. While asymptomatic and mildly symptomatic patients can be managed non-operatively, overtly symptomatic patients should have the affected lobes resected. We present the case of a 19 month old female with symptomatic right-sided congenital lobar emphysema resulting in mass effect and leftward displacement of the heart and mediastinal structures with compressive atelectasis of the left lung. Therefore, resection of the affected lobes was indicated. During resection we identified a distinct margin between normal lung tissue and diseased lung tissue within a single lobe, and successfully performed a wedge resection of the diseased portion as opposed to removing the entire lobe. In our video we demonstrate that thoracoscopic wedge resection of congenital lobar emphysema is technically feasible even in severe disease.