Abstract:Primary sarcomas constitute only 1-2% of malignant renal tumours in adulthood and of these leiomyosarcomas account for 50-60%. We describe here a case of renal leiomyosarcoma in an elderly woman, presenting with abdominal lump, anorexia and weight loss for 3 months. Based on CECT abdomen findings, a presumptive differential diagnosis of sarcomatoid variant of renal cell carcinoma/ renal sarcoma was made. Microscopy reflected a malignant spindle cell neoplasm which showed strong reactivity for smooth muscle actin with negative epithelial markers, thereby confirming the diagnosis of renal leiomyosarcoma. To conclude the entity of renal sarcoma should be taken in to consideration while evaluating renal tumours, particularly in older females, owing to their aggressive nature and low survival rates.Key words: Leiomyosarcoma, Kidney neoplasms, Sarcoma, Anorexia, Weight loss, Diagnosis.