Abstract
Acute renal haemorrhage is a life-threatening condition that is complicated in the context of renal malignancy. Here, we present the case of a teenage male presenting acutely with a large, bleeding renal epithelioid angiomyolipoma (EAML) of the kidney-a rare cancer, which is part of the perivascular epithelioid cell tumour family. The patient was managed acutely with prompt resuscitation, transfer to a centre of expertise and haemorrhagic control using radiologically guided endovascular techniques; this subsequently permitted an oncologically sound procedure (radical nephrectomy, inferior vena cava thrombectomy and lymphadenectomy) to be performed within 24 hours. The description and discussion around this unique case summarises the patient's clinical journey, while exploring the current literature surrounding diagnosis and outcomes of patients with renal EAMLs.