The 2022 WHO classification introduced several new molecularly defined categories of RCC, including those with TFE3 rearrangements, TFEB rearrangements, TFEB amplification, FH deficiency, SDH deficiency, ALK rearrangements, ELOC mutations and SMARCB1 (INI1) deficiency RCC (1, 2). Here, SMARCB1 is linked to hyperinsulinemic hypoglycemia, familial, 4.