Insulin-secreting tumors are the most common hormones, producing neoplasms of the gastrointestinal tract. Among them, only 10% of insulinoma is malignant. We report a rare case of malignant insulinoma in a 58-year-old female who presented with recurrent episodes of hypoglycemia and symptoms of neuroglycopenia, such as recurrent generalized tonic-clonic seizures. Evaluation revealed low fasting and random blood sugars with high levels of serum C-peptide, proinsulin, and insulin. Radio-imaging revealed mass in the head of the pancreas and multiple lymph nodal and liver metastases. Her symptoms were refractory to medical therapy with somatostatin analogs and were requiring frequent parenteral dextrose boluses. She was then subjected to the resection of mass and liver metastasectomy, following which addition of everolimus to somatostatin analog dramatically abolished her symptoms. She is symptom-free for the last 11 months on somatostatin analog and everolimus.