Giant carotid body tumors, defined as those >8 cm in size, are extremely rare. Definitive surgical management is a complex undertaking because these large tumors tend to have grown to envelop cranial nerves and the carotid artery, and few data exist regarding the long-term outcomes for these patients. We present the case of a patient with bilateral giant carotid body tumors who underwent staged embolization and excision of a >10-cm carotid body tumor. After 5 years of follow-up, we demonstrated that elective open repair can provide long-term symptomatic relief. We describe and illustrate the crucial steps and considerations regarding the excision of complex Shamblin type III carotid body tumors.