While venous thromboembolism (VTE) in children with nephrotic syndrome (NS) remains an uncommon clinical entity, it represents one of the disease's most severe and potentially fatal complications. As such, clinicians and radiologists must maintain a high level of suspicion for VTE and low threshold for performing diagnostic imaging studies in children with NS, thereby ensuring prompt diagnosis and early management initiation. Despite the recent advances and development of image-guided endovascular procedures, there remains a marked paucity of literature describing the use of endovascular intervention for the treatment of acute VTE in NS, and a clear consensus on the gold standard for management has yet to be fully elucidated. Moreover, given the relative rarity of this complication in children as opposed to adults, no prior report has been made in which a paediatric patient has undergone endovascular intervention for acute VTE in the setting of NS. This report will outline the use of endovascular pharmacomechanical thrombolysis and thrombectomy as a novel treatment option for acute circumaortic left renal vein and inferior vena cava thrombosis in a paediatric patient with relapsing NS.