Insulinomas are rare tumors with an estimated incidence of one per 250,000 person-years. Most insulinomas are benign with less than 10% demonstrating malignant behavior, the vast majority of which occur in adults. A systemic review of the literature revealed only nine cases of malignant insulinomas occurring in children. Herein, we present a case of metastatic malignant insulinoma in a 12-year-old child. The occurrence of this diagnosis in a child, its unusual pattern of metastases and the challenging management of severe hypoglycemia make this case worth reporting.