Human herpesvirus 8 (HHV-8), also known as Kaposi's sarcoma (KS)-associated herpesvirus, is the etiologic agent responsible for all types of KS. Although the majority of pediatric KS cases occur in sub-Saharan Africa, a rise in pediatric transplant KS has been reported in developed countries. In addition, HHV-8 is increasingly described as an infectious cause of hemophagocytic lymphohistiocytosis in children. Transmission of HHV-8 among children is poorly understood; however, the literature strongly suggests that horizontal transmission plays a critical role. Acute infection with HHV-8 and progression to KS in children may be different than in adults, and diagnosis may be overlooked. Currently, neither adult nor pediatric treatment guidelines exist. This review provides an overview of HHV-8 disease in children as it relates to epidemic KS, transplant KS, and other disease manifestations. The current state of the literature is reviewed and knowledge gaps are identified for future exploration.