Carotid body tumor (CBT), an extra-adrenal paraganglioma, represents an uncommon entity arising in chemoreceptor tissue located at the carotid bifurcation. Only a few cases have been reported in the literature in the pediatric age group less than 14 years of age, primarily as case reports. Surgical excision is advisable in almost all ages, however, in childhood, it is known to carry risks and difficulties due to the smaller size of the involved anatomical structures. We report an unusual case of a large (50 x 43 x 30 mm) carotid paraganglioma of Shamblin type III in a 13-year-old female, living at high altitude until the age of 9 years. This cervical mass was present, but smaller in size, several years before admission. However, an intense hypoxic stimulus at high altitude for 2 months at the age of 13 years probably induced a fast growing period of the lesion. The tumor was removed en bloc with the involved carotid segments and vascular continuity was restored by means of a common carotid to internal carotid saphenous vein interposition graft. No malignant behavior or regional metastatic spread of the resected mass was demonstrated. Furthermore, no inheritance pattern between family members was confirmed. A clinical update on CBTs in childhood a propos of this case report is undertaken.