Solid pseudopapillary neoplasms (SPNs) are the most common pediatric pancreatic tumor; however, most data in children are extrapolated from adults. This study describes a young presentation of SPN in a 5-year-old girl and presents a comprehensive systematic review of the literature regarding SPNs in children. A systematic review was performed using PubMed and Embase for all articles in English using predetermined search terms, including “solid pseudopapillary neoplasm” and “pediatric” and historical terms for SPN. A total of 523 pediatric patients were identified in 135 articles. Eighty-three percent of patients were female, and median age was 13.6 years. Abdominal pain was the most frequent presenting symptom (78%), and median tumor size was 8.2 cm. The pancreatic head was involved in 46% of cases. Computed tomographic scan was the most common imaging modality (87%), and 61% were diagnosed by fine needle aspiration. Surgical resection was reported in 507 patients, with a complication rate of 21.1% reported in 393 patients. Only 3.8% received adjuvant therapy, and 6.7% had recurrent disease. Solid pseudopapillary neoplasms of the pancreas are rare tumors in childhood. Male sex and pancreatic head involvement are seen more often in children than in adults. Surgery remains the mainstay of treatment with excellent results.