A case of nodular sclerosis Hodgkin's disease, stage IVB, with lung involvement and hypertrophic pulmonary osteoarthropathy (HPOA), was treated with quadruple cytotoxic chemotherapy. The pulmonary lesions, which were thin walled cavities, and the lymphadenopathy resolved completely after two courses of chemotherapy. The clubbing and all evidence of periosteal new bone formation disappeared after six courses of treatment. Complete reversal of the syndrome by chemotherapy has not been previously described. A literature review revealed 13 more cases of Hodgkin's disease associated with HPOA. The syndrome occurred for the first time at the time of diagnosis of Hodgkin's disease in 11 cases, and at recurrence in two. Tha majority of patients had advanced disease (Stage IIIB or IV). Mediastinal involvement was present in all 12 cases for which data were given; lung involvement was present in six cases and pleural involvement in three. Three cases were of the nodular sclerosis type, and one of the lymphocyte-depletion type. The HPOA syndrome in young patients with malignancy is indicative of tumors other than primary bronchial carcinomas.