We report a case of fatal aortic tumor embolism presenting as acute paraplegia. A four-year-old girl was referred from a local hospital with sudden paraplegia and a poor medical condition. A neighbor had noticed her fall from a bike, and she could not walk. She had no previous illness. Emergency spine MRI revealed no remarkable findings. During the process of evaluation, her general condition deteriorated progressively. Chest and abdominal CT showed a large mass in the left lung field, and a diagnosis of aortic occlusion was made. An emergency transfemoral embolectomy was attempted. However, the patency of the aorta was not recovered. On pathological examination of tissues taken from the embolectomy, a pleuro-pulmonary blastoma was found. The patient died 22 hours after the onset of her symptoms. We describe a possible mechanism for the tumor embolism. To the best of our knowledge, this is the first case report of aortic occlusion caused by an embolic malignancy, presenting as acute paraplegia.