Pheochromocytoma is a rare tumor originating from neuroectodermic cells. Only 10% of these tumors are malignant. There are many familial forms of this tumor, including multiple endocrine neoplasia type II, Von Hippel-Lindau syndrome, and neurofibromatosis type I. Skin manifestations of pheochromocytoma are rare, and cutaneous metastasis in patients with multiple endocrine neoplasia IIB has never been described. The case of a patient with multiple endocrine neoplasia IIB who presented malignant pheochromocytoma with multiple cutaneous metastasis is described.