Congenital lobar emphysema (CLE) is a life-threatening but potentially reversible lung abnormality of unknown etiology that can present a diagnostic dilemma. Imaging procedures are helpful for early diagnosis. This paper aims to present the scintigraphic features of CLE in a two-year-old male in correlation with other diagnostic modalities. Lung perfusion scintigraphy revealed globally diminished perfusion to the left lung. High Resolution Computed Tomography (HRCT) of the chest showed intrinsic narrowing of the distal left mainstem bronchus. CT scan of the Pulmonary Arteries (CTPA) showed a small left main pulmonary artery. Lung perfusion scintigraphy in CLE is characterized by markedly attenuated vascularity of the involved lobe with consequent decreased perfusion, and increased to normal perfusion in the unaffected lobe. It plays a vital role in the early diagnosis and management of pediatric pulmonary diseases presenting with a diagnostic dilemma, particularly in cases where it can preclude more invasive diagnostic procedures.