A 17-year-old girl presented with a 3 month history of alopecia areata, a generalized scaling skin, enlargement of the inguinal lymph nodes and severe back pain. Staging procedures revealed multifocal bone disease and generalized lymphadenopathy. The diagnosis of nodular sclerosing Hodgkin's disease was established by biopsies of the os ileum and a left inguinal lymph node. Complete clinical remission was achieved after 2 OPPA (vincristine, prednisone, procarbazine, and doxorubicin) and 4 COPP (cyclophosphamide, vincristine, prednisone, and procarbazine) cycles and treatment completed with involved-field irradiation. After the completion of therapy, skeletal lesions had mostly resolved or become sclerotic and the patient had normal hair regrowth and skin appearance. Conclusively, this case illustrates that alopecia areata may occur as a paraneoplastic phenomenon or an autoimmune process related to the deranged cellular immune system in children and adolescents with Hodgkin's disease.