Introduction: Angiomyolipoma (AML) also known as hamartoma is a rare benign tumour composed of an admixture of adipose tissue, smooth muscle and abnormal blood vessels. AMLs of the kidney are seen in 20% of patients with tuberous sclerosis. In an enlarged form, an angiomyolipoma mimics a renal cell carcinoma, therefore it is imperative to rule it out. Surgery management is recommended for confirmatory histological diagnosis and additionally helps by preventing the risk of haemorrhage or malignancy. In our case, we report a 48-year-old female with complaints of lump and pain in the abdomen. Case Report: Contrast-enhanced Computed Tomography (CECT) was suggestive of left renal mass with large cystic lesion over the right ovary. The patient was taken for exploratory laparotomy and a left nephrectomy with right oophorectomy was carried out. On histopathological examination, renal angiomyolipoma with serous cystadenoma of the ovary was seen. Conclusion: Renal angiomyolipoma by itself is a rarity. Therefore, the simultaneous occurrence of a renal AML with serous cystadenoma of the ovary makes us wonder whether this is an isolated incident or a part of a genetic complex yet to be discovered.