ABSTRACT We report a 5-year boy presenting with pain in the lower chest and upper abdomen. On evaluation with computed tomography scan of the chest, he was found to have a large 16 x 14 cm posterior mediastinal mass compressing the inferior vena cava and liver, and shifting the heart to the opposite side. Tumor was approached via right postero-lateral thoracotomy and dissected off from esophagus, heart, inferior vena cava and lungs. The entire tumor was resected without any complications. Postoperative course was unremarkable. Resection of the tumor was successful. The histopathology showed it to be giant ganglioneuroma with no evidence of malignancy.