Hodgkin lymphoma is exceedingly rare in children, as is primary orbital manifestation of systemic Hodgkin lymphoma in any age category. The authors describe the clinicopathologic manifestations of a rare case of systemic Hodgkin lymphoma with primary orbital manifestation in a 3-year-old boy with histologically proven Hodgkin lymphoma with mixed cellularity. After an incisional biopsy and tumor debulking, followed by systemic workup, the diagnosis of stage IVA Hodgkin lymphoma was made. He underwent treatment with 4 cycles of adriamycin, bleomycin, vincristine, etopiside, prednisone, and cyclophosphamide, followed by involved field radiation. He has been followed at regular intervals for 6 years. Scans have shown resolution at all sites, and blood counts have remained normal. To the best of the authors' knowledge, this is the youngest patient with primary orbital manifestation of Hodgkin lymphoma reported in the English literature.