Genetic kidney diseases are common, accounting for up to 20-40% of severe cases in young patients. Identifying them helps avoid unnecessary immunosuppressive treatments, tailor clinical management, anticipate extra-renal complications, and provide genetic counseling. The main suggestive situations include: a treatment-resistant Focal Segmental Glomerulosclerosis (FSGS), an early-onset hematuria or a proteinuria, the structural abnormalities, the syndromic manifestations, or a family history of kidney disease. The increasing use of high-throughput sequencing (exome, gene panels, genome) improves diagnostic yield and guides follow-up, family screening, and the assessment of eligibility for living kidney donation among potential donors within a given family.