Epithelioid angiomyolipoma is a rare variant of renal tumour with potential for aggressive biological profile. We present a12 year old girl with a large angiomyolipoma that created diagnostic and treatment difficulty and review this condition. A 12 year old girl presented with right flank pain. Imaging discovered a large mass arising from the upper pole of the right kidney but inseparable from the adrenal gland and under-surface of the liver. The lesion was highly vascular with a very large draining vein into the renal vein, initially precluding safe needle biopsy. MRI showed small component of fat. A provisional diagnosis of angiomylipoma was made. There was no systemic evidence of tuberous sclerosis. The patient required multiple episodes of embolization to enable safe biopsy as there was diffuse dysplastic and aneurysmal arterial supply from renal, adrenal, phrenic, lumbar and hepatic arteries. Biopsy was performed percutaneously with a balloon in the renal artery. Histology confirmed epithelioid angiomyolipoma. Further sequential embolization was performed to devascularise the tumour prior the surgery. The patient was able to undergo a partial nephrectomy with virtually no blood loss. Epithelioid angiomyolipoma is a rare variant of angiomyolipoma which has been described to have a more aggressive course in some patients. They tend to be larger lesions. The paucity of fat in our patient made imaging diagnosis doubtful. Embolization not only made biopsy safer but also facilitated a safe partial resection of the affected kidney. Histology confirmed the initial diagnosis with complete resection of the lesion. Epithelioid angiomyolipoma defines a group of tumours that are not potentially malignant. This case shows that biopsy of vascular lesions and renal sparing surgery is possible and safe when combined with pre-operative embolization. We highlight the role of pre-operative embolization to avoid haemorrhage and facilitate safe dissection of operative planes.