A patient with nodular sclerosing Hodgkin's disease and basal cell carcinoma syndrome is reported. Medulloblastoma and ovarian fibromas have already been associated with the syndrome and its coexistence with other malignant neoplasms has been described. The authors report here the first case of Hodgkin's disease with the multiple nevoid basal cell carcinoma syndrome. The fact that the patient's mother also has the syndrome, and the maternal grandfather had Hodgkin's disease seems to indicate more than a chance association of two independent events.