INTRODUCTION: Tuberous sclerosis complex (TSC) is an autosomal dominant disease that affects multiple organs. Medical therapy with the mTOR inhibitor everolimus has become the first option in patients with angiomyolipomas. But mTOR inhibitor treatment shows no effect in some patients, in the case, surgery is a suitable method for treatment. PRESENTATION OF CASE: A 30-year-old Chinese male patient received an ultrasound examination of the kidney, which showed bilateral hyperechogenic structures without stones or ureterohydronephrosis. A computed tomography (CT) scan of the kidneys showed multiple slightly high-density masses (largest size: left 6.5 cm, right 5.2 cm), and the masses lacking of lipids were obviously enhanced in the arterial phase. To clarify the components of the tumors, we performed retroperitoneal laparoscopic tumor enucleation to remove all visible masses in left kidney. The pathological results and genetic tests confirmed the diagnosis of TSC. For reducing the further spontaneously bleeding risk and the cost burden, the right renal tumors were also enucleated. Twelve months after the second operation, renal function remained normal, and no tumors were detected by CT. DISCUSSION: Large angiomyolipomas (>4 cm in diameter) may develop life-threatening hemorrhage or compress normal kidney tissue. Fat-poor renal angiomyolipomas are difficult for making a differential diagnosis from renal cancer or renal sarcomatoid carcinoma. When medication treatment does not work, surgery is a good option to diagnose and treat big bilateral renal angiomyolipomas. CONCLUSION: After 12 months of follow-up, retroperitoneal laparoscopic tumor enucleation may be a safe and effective method for treating big fat-poor renal angiomyolipomas in patients with TSC.