Since DICER1 syndrome predisposes affected individuals to multiple neoplasms throughout their lifetime, the International DICER1 Symposium recommends surveillance strategies for DICER1-associated pulmonary, renal, gynecologic, thyroid, ophthalmologic, otolaryngologic, and central nervous system tumors and gastrointestinal polyps. The gene discussed is DICER1; the disease is DICER1-related tumor predisposition.