Pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors leading to serious complications in the cardiovascular system. As somatostatin receptor (SSTR) is highly expressed in PPGL, SSTR-targeting imaging, particularly PET/CT based on ⁶⁸Ga-labelled somatostatin analog represented by ⁶⁸Ga-1, 4, 7, 10-tetraazacyclododecane-1, 4, 7, 10-tetraacetic acid-D-Phe1-Tyr3-Thr8-octreotide (DOTATATE), becomes an important tool for location and assessment of systemic metastases. Treatments for metastatic PPGL are limited. Peptide receptor radionuclide therapy (PRRT) with ¹⁷⁷Lu-DOTATATE provides a new therapeutic option for patients with inoperable PPGL and demonstrates satisfying efficacy. This article summarizes the advances of SSTR-targeting imaging and PRRT in the diagnosis and treatment of PPGL.