Angiomyolipoma is a rare benign tumour of the kidney. They are composed of abnormal vasculature, smooth muscle and adipose elements. They may be associated with Tuberous sclerosis. The unilateral presentation is uncommon. Angiomyolipoma is presented as an enlarge lump in the abdomen may mimic malignant lesion such as renal cell carcinoma, yet malignancy has to be ruled out. The diagnosis of this condition may not be straight forward with imaging alone. Excision is recommended for definite histopathological diagnosis (in symptomatic patients) and to prevent the potential risk of haemorrhage and malignancy. A case of benign giant Angiomyolipoma is presented here because of its uncommon occurrence.