Abstract: Tuberous sclerosis-complex renal angiomyolipoma (TSC-RAML) is a rare benign tumor composed of different proportions of fat, blood vessels and smooth muscle tissue. The current clinical treatment methods include mTOR inhibitors, selective arterial embolization and surgery. Imaging examination provides important references for the diagnosis, treatment and follow-up monitoring of TSC-RAML, but the results of computed tomography (CT) and magnetic resonance imaging (MRI) are subject to subjective differences due to the influence of clinical experience. Two-dimensional images with defects such as non-intuitive, non-stereoscopic and unable to be integrated into a whole are easy to cause diagnosis and treatment deviation. In recent years, the emergence of three-dimensional reconstruction provides an objective reference for a full understanding of the disease, reasonable preoperative planning and accurate operation. In addition, three-dimensional images are clear and intuitive, which makes it widely used in clinic. The application of three-dimensional reconstruction in the treatment of TSC-RAML is reviewed in this paper.