Though rare, angiomyolipomas (AMLs) are the most common mesenchymal tumors of kidney. In general, AMLs can always be associated with two conditions affecting other organ systems: tuberous sclero-sis complex (TSC) and sporadic lymphangioleiomyoma-tosis. This article presents a case of renal AML occurring in a 14-year-old girl with a definite diagnosis of TSC. She had been diagnosed with TSC at the age of three, and a schedule for close observation was disobeyed. At this time, she underwent a series of examinations: physical examina-tion, ultrasonography, angiography, computed tomography (CT), and magnetic resonance imaging (MRI) scans. The physical examination showed adenoma sebaceum in a butterfly paranasal distribution, and a mass was palpated in the left upper quadrant. There were no neurological deficits. Imaging studies (including ultrasonography, angiography, CT, and MRI) of the abdomen showed a large heterogeneous mass arising from the left kidney. Partial nephrectomy was performed. The pathological diagnosis was hemorrhagic renal AML. No recurrence was found in the three-year follow-up. We concluded that schedule of close observation on patients with TSC should be strictly abided by for the high morbidity of AMLs. The specific risks of renal AMLs are spontaneous hemorrhage and rupture. Treatment options for AMLs include conservative and interventional (total/partial nephrectomy, cryoptherapy, and embolization) treatments.