A better understanding of positron emission tomography (PET) response in nodular lymphocyte-predominant Hodgkin lymphoma (nLPHL) is critical for incorporating PET into prospective trials. PET scans from Children's Oncology Group study AHOD03P1 for patients less than 22 years with low-risk nLPHL, treated with three cycles of doxorubicin, vincristine, prednisone, and cyclophosphamide chemotherapy, were retrospectively reviewed and assigned Deauville 5-point scale (5PS) scores. Five-year post-PET event-free survival was 90.1% (80% CI: 85.2%-93.4%) for PET-negative (5PS 1-3) and 66.7% (80% CI: 36.4%-85.0%) for PET-positive (5PS 4-5) patients. PET response after three cycles of low-dose chemotherapy is predictive of relapse risk for low-risk nLPHL.