Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by the presence of proliferative lesions throughout the body. Management of TSC is challenging because patients have a multifaceted systemic illness with prominent neurological and developmental impact as well as potentially severe kidney, heart and lung phenotypes; however, every organ system can be involved. Adequate care for patients with TSC requires a coordinated effort involving a multidisciplinary team of clinicians and support staff. This clinical practice recommendation was developed by nephrologists, urologists, paediatric radiologists, interventional radiologists, geneticists, pathologists, and patient and family group representatives, with a focus on TSC-associated kidney manifestations. Careful monitoring of kidney function and assessment of kidney structural lesions by imaging enable early interventions that can preserve kidney function through targeted approaches. Here, we summarize the current evidence and present recommendations for the multidisciplinary management of kidney involvement in TSC.Sections A preliminary search was conducted in one of the biomedical core databases (MEDLINE or Embase). Information specialists contacted the working groups to clarify terms and achieve a balanced search regarding specificity and sensitivity as well as to decide whether to consult systematic reviews or clinical trial databases like Database of Abstracts of Reviews of Effects (DARE), Cochrane Database of Systematic Reviews (CDSR), WHO International Clinical Trials Registry Platform (ICRTP),