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Carotid body tumors (CBTs), or chemodectomas, are rare, especially in the pediatric population. They often present with minimal symptoms, making timely diagnosis challenging. This case report and systematic review highlights a distinctive presentation and summarize the current evidence on pediatric CBTs. We report a case of a 13-year-old girl presenting with neck pain and a left-sided neck mass. After extensive evaluation, a Shamblin type III tumor was identified and removed surgically. Postoperatively, the patient experienced transient hypertension and significant dysphagia, both of which resolved within a few weeks with no permanent sequelae. Histology confirmed a benign paraganglioma. A systematic literature review of PubMed identified 29 cases from 23 published studies spanning from 1968 to 2024. The average age at diagnosis was 12.6 ± 3.6 years. The most common symptom was a neck mass or swelling, reported in 75% of cases (n = 21). Tumor sizes ranged from 1.3 to 8.0 cm, with Shamblin III being the most frequent classification. Gross total resection (n = 25 [89.3%]) alone or in combination with preoperative embolization (n = 10 [35.7%]) were the most common methods of management. In 62.1% of cases, there were no permanent complication or sequelae. The proximity to vital neurovascular structures and high vascularity in pediatric patients necessitates careful perioperative interdisciplinary management. Owing to their rarity and nonspecific presentation, CBTs often remain undiagnosed for years. They respond well to treatment, but can be fatal if untreated, underscoring the importance of including CBTs in the differential diagnosis of pediatric neck masses.