Abstract
Angiomyolipoma is a benign mesenchymal tumour of kidney that consists of adipose tissue, muscle cells and blood vessels. Renal angiomyolipomas represent almost one percent of all renal tumours. We reported a case of a 50-year-old woman complaining of mild abdominal discomfort with no other symptoms and no remarkable medical history. Clinical examination was inconclusive and no lump was palpable in abdomen. Ultrasonography raised the suspicion of existence of a large, homogeneous, hyperechoic tissue mass arising from right kidney. Abdominal computed tomography scan suggested the presence of giant angiomyolipoma of right kidney. The histopathological examination confirmed the diagnosis of renal angiomyolipoma. The patient underwent open simple nephrectomy to remove the tumour without any complications. We documented a rare case of retroperitoneal angiomyolipoma with extrusion of fat content from the renal hilum breaching the cortex as peculiarity which presented just as mild abdominal discomfort.