While our patient's hypoglycemia occurred in the absence of IGF-II testing, nonsuppressed insulin-related markers and resolution post-adrenalectomy suggest a different, likely catecholamine-mediated mechanism rather than NICTH. This rare phenotype may result from a combination of reduced insulin resistance in NF1 patients and catecholamine-induced metabolic alterations leading to the intermittent dysregulation of glucose homeostasis, potentially unmasking subclinical hypoglycemia. Here, INS is linked to neurofibromatosis type 1.