Out of 33 pediatric ACTH-dependent Cushing’s syndrome cases investigated at CMHI between 1993 and 2020, only 2 had ectopic ACTH syndrome (neuroendocrine tumor of the thymus and appendix), which corresponds to 6.5%, none of whom had BIPSS performed (30). The gene discussed is POMC; the disease is Cushing syndrome due to macronodular adrenal hyperplasia.