Case report: A 67‐year‐old female had a solid renal mass picked up incidentally on computed tomography (CT) for investigation of bloating. It was an enhancing lesion on a contrast CT scan. Decision was made to proceed with a partial nephrectomy given the suspicions this was a renal cell carcinoma. The patient was asymptomatic on review and had no past medical history. The specimen was 25 × 22 × 12 mm. Microscopic sections from the specimen showed scatted single cell positivity for HMB45 present, indicative of LA from the tissue diagnosis demonstrated. Her post‐operative care was unremarkable. She will be followed up 6 monthly surveillance imaging.

Discussion: On CT, LA shows a characteristic solid appearance due to the fact they have abundant adipose tissue. This poses diagnostic difficulty as they appear highly suspicious for renal cell carcinoma. Microscopically, LA is filled with spindle cells of smooth muscle type that may look like leiomyomas, leiomyosarcoma, or gastrointestinal stromal tumors. The presence of perivascular epithlioid cells in tumour tissue, show reactivity to melanocytic markers such as HMB‐45 which confirm the diagnosis of LA.

Conclusion: Leiomyomatous angiomyolipomas are extremely rare renal lesions with only two cases reported in the literature. They are benign lesions which are characterised by HMB‐45 on immunostain. Our case adds to the limited literature on this rare pathology.