Purpose Sclerosing angiomyolipomas (AMLs) are a subtype of perivascular epithelioid cell-omas (PEComas). Sclerosing AMLs/PEComas are predominantly benign, retroperitoneal, and are overwhelmingly reported in females. We report a renal sclerosing AML/PEComa in a male to highlight the importance of considering sclerosing AMLs/PEComas as an indolent differential for small renal masses in men, as accepted knowledge of small renal masses with these characteristics suggests 70-80% will be malignant. Methodology Case selection included patients at Royal North Shore Hospital with renal sclerosing PEComas/AMLs. Results A 56-year-old man with a 17 mm left lower pole Bosniak III renal lesion underwent partial nephrectomy without complication. Microscopically, the lesion was well defined from the renal cortex and extended into the perinephric fat. Largely uniform cells with round-to-oval nuclei were arranged in cords, surrounded by sclerotic collagenous stroma. There was no evidence of tumour necrosis and mitotic figures were inconspicuous. Immunohistochemistry was positive for desmin, smooth muscle actin, and human melanoma black-45. These features suggested a sclerosing AML/PEComa without atypical features or epithelioid characteristics. The patient remains free of recurrence or metastasis five months post-operatively. Conclusions Sclerosing AMLs/PEComas are an important differential for small renal masses in men. Sclerosing AMLs/PEComas can be differentiated from potentially malignant pathologies with immunohistochemistry. By highlighting the appearance of this benign lesion in men, we highlight sclerosing AMLs/PEComas as an important benign differential for small renal masses in this group.