<h4>Introduction</h4>Although bilateral pheochromocytoma is prevalent in patients with multiple endocrine neoplasia type 2, extra-adrenal tumors rarely occur in the aortocaval area.<h4>Case presentation</h4>A 35-year-old man with multiple endocrine neoplasia type 2A (<i>RET</i> codon Cys634Arg mutation) underwent bilateral adrenalectomy for metachronous pheochromocytoma. Here, RET is linked to hereditary pheochromocytoma-paraganglioma.