Skeletal muscle involvement in Hodgkin lymphoma is very rare. An 11-year male child presented with stage IV Hodgkin lymphoma and skeletal muscle involvement in right gluteal, piriformis and psoas muscles. He had resistant disease. He achieved remission with 3rd line chemotherapy, EPIC; and successfully underwent high dose chemotherapy with autologous stem cell rescue. The patient is well after 15 months of follow-up. Successful treatment signifies positron emission computed tomography (PET/CT) evaluation and multidisciplinary team discussions in diagnosis and management of a rare Hodgkin lymphoma presentation.