"Vascular leiomyoma originating in the renal vein is a rare entity. Our case allowed us charting of progression of renal vein leiomyoma for 8-years’ duration. Apart from intraluminous growth causing displacement of viscera, the leiomyoma did not show any aggressive behaviour. Eventually, removal of the tumour through nephrectomy provided complete symptomatic relief to our patient. A 75-year old female presented with an eight-year long history of severe pain in right flank. CT imaging revealed a mass arising from the right renal vein and a few hepatic tumours. The differentials included leiomyosarcoma, in view of hepatic lesions. Excision of the tumour was performed with radical nephrectomy. Immunohistopathological examination confirmed it to be a case of leiomyoma. Three months post-op, the patient reported complete pain relief and no adverse events, and there were no signs of recurrence. Learning Points: 1) Vascular leiomyomas do not show any aggressive behaviour. However, their growth tends to displace the normal viscera, causing pressure effects that may give rise to intractable pain. 2) Vascular obstruction affects GFR and renal function. In our patient, despite a long period of growth, the tumour did not show any malignant changes. 3) Tumour excision through radical nephrectomy resulted in symptomatic improvement in our patient. Since imaging alone does not distinguish a leiomyoma from its malignant counterpart with certainty, surgical excision may be warranted in all the cases. Source: https://www.sciencedirect.com/science/article/pii/S2210261219306145"