Sterile pyuria is a common finding in pediatric patients. Literature describing the diagnoses as well as clinical characteristics of children with sterile pyuria is lacking. This review was performed to establish an evidence-based approach to the differential diagnosis by way of an extensive literature search. The definition of pyuria is inconsistent. The various causes of pediatric sterile pyuria identified were classified as either Infectious or Non-Infectious. Sub-categories of Infectious causes include: Viral Infection, Bacterial Infection, Other Infections (tuberculosis, fungal, parasitic), Sexually Transmitted Infections, Recent Antibiotic Therapy. Non-Infectious causes include: Systemic Disease, Renal Disease, Drug Related, Inflammation adjacent to Genitourinary Tract. Clinicians that encounter pediatric patients with sterile pyuria and persistent symptoms should consider the substantial differential diagnosis described in this study.