We report a case of a 13-year-old boy who complained of progressive abdominal distension and symptoms of anaemia. Radiological investigations revealed that the child had a hypervascular tumour of the inferior vena cava (IVC). Unfortunately, the child presented with acute lower gastrointestinal bleed soon after the investigation. He underwent an urgent pre-operative embolisation, aimed to reduce the tumour vascularity. A total resection of the tumour, right nephrectomy, and partial duodenal resection were done within 24 hours post-embolisation. The child was stable postoperatively. The histopathological examination revealed chromogranin-positive paraganglioma originating from the IVC. We highlight the radiological findings of rare primary IVC paraganglioma and the role of embolisation prior to surgical removal of the tumour.