Background Progressive transformation of germinal centers (PTGC) is a rare diagnosis characterized by asymptomatic lymph node enlargement. It has previously been associated with lymphoma, autoimmune conditions, and lymphoproliferative diseases in small pediatric case series. Procedures We conducted a single-center retrospective review of pediatric cases of PTGC diagnosed at our institution by hematopathologists from 2000 - 2020. Results We identified 57 primary cases and 3 recurrent cases of PTGC. There was a male predominance in cases (32/57), with median age at diagnosis of 11 years. Head and neck lymph nodes were the most commonly involved and biopsied sites. Laboratory and imaging evaluations were obtained inconsistently. Only 16% of patients saw a pediatric hematology/oncology (PHO) specialist prior to diagnosis and 37% had follow-up with PHO after diagnosis. Six patients (10%) had a preceding or concurrent diagnosis of lymphoma and 5% of patients returned with recurrent PTGC. Conclusions Patients with PTGC had similar characteristics to those from previous case series. Fewer patients underwent recurrent lymph node biopsy than previously described. PTGC has been linked to certain types of lymphoma, although never definitively associated with lymphoma. Follow-up with a PHO provider is indicated to ensure that close surveillance is performed.