Angiomyolipomas are benign mesenchymal tumours originating from the kidney and adrenals. They are rare tumours that can be sporadic and isolated or occur as a part of tuberous sclerosis. These tumours have a high content in the cells, which is pathognomonic for diagnosis using ultrasonography, computed tomography and magnetic resonance imaging. Atypical angiomyolipomas occur with excessive smooth muscle cells and less adipose tissue, and are sensitive to immunohistochemistry studies. Most of these lesions are detected incidentally but some can cause back and abdominal pains if large in size. Larger lesions are also vulnerable to spontaneous or traumatic rupture, causing large retropertitoneal bleeds. Surgery should be considered as the definitive management for larger lesions to avoid associated complications. There have been no reports of any malignant change being reported in any of the lesions but a long follow-up period is still required, given the unknown clinical progression of these rare tumours.