Adrenal cortical carcinoma (ACC) is a rare invasive cancer with a poor prognosis. ACC often manifests with hyperglycemia and Cushing syndrome. Hypoglycemia is an unusual manifestation of this malignant tumor; therefore, the diagnosis and management of patients with ACC and hypoglycemia is extremely challenging. The current study presents the case of a 53-year-old woman who experienced recurrent, uncontrolled hypoglycemia and episodes of hypoglycemic coma. The patient was diagnosed with right-sided ACC and successfully treated with surgery. Hypoglycemic episodes associated with ACC are extremely rare. Moreover, the clinical diagnosis is often challenging owing to its similarity to multiple other diseases. Recurrent severe hypoglycemic comas, if not addressed promptly, can lead to patient death. Therefore, it is necessary to monitor patients with such rare diseases in clinical practice and make an accurate diagnosis promptly to ensure timely and effective treatment. This report of an ACC case with non-islet cell tumor hypoglycemia aims to raise awareness among clinical physicians.