Abstract DICER1 syndrome is a hereditary tumor predisposition syndrome caused by pathogenic germline variants in DICER1 and is characterized by multi-organ susceptibility; sarcomas remain uncommon within this spectrum. We report a 2-year-old girl with anaplastic sarcoma of the kidney (ASK). Molecular testing identified a germline DICER1 c.3300dupA frameshift variant (p.S1101Ifs*3), inherited from her father, and a tumor-associated c.5125G>A missense variant (p.D1709N).
