Thyroid follicular carcinoma like renal tumour (TFCLRT) is a rare variant of primary renal epithelial tumour and was first reported in 2006. Up till now, 40 cases have been identified worldwide and alarmingly, 17 cases have been identified from China only. The condition has been included in the WHO Renal Tumours Classification 2016. We present here the first case of thyroid follicular carcinoma like renal tumour from Pakistan that was managed in our surgical unit and a literature review. Left-sided radical nephrectomy was performed through a midline incision. The left kidney was removed along with intact Gerota fascia, left adrenal gland and lymph nodes alongwith aorta.