Carotid body tumors (CBTs) are rare neoplastic paragangliomas that are typically benign; however, 10% to 15% are malignant with local invasion and, in rare cases, metastases systemically. A 45-year-old woman with a familial history of CBT presented following a syncopal episode. Multimodal imaging confirmed the presence of bilateral CBTs. 68Gallium-DOTATATE Positron Emission Tomography (DOTATATE-PET) scans revealed distant systemic metastases in the liver and vertebra. Surgical intervention was undertaken to excise the less complex right CBT. This report presents a case of malignant CBT with systemic metastases, describing the postintervention multimodal imaging findings with a focus on the duplex ultrasound features.