We have described the first case, to our knowledge, in which recurrent respiratory tract infections were the primary manifestation of thymoma with immunodeficiency (Good's syndrome) associated with cobalamin malabsorption and immunoglobulin M-kappa (IgM-kappa) M component. The intrinsic factor receptor activity was dramatically decreased in a mucosal homogenate prepared from ileal biopsies. This decreased activity could be the principal cause of the malabsorption of labelled cobalamin which was observed in the presence of intrinsic factor. However, it could be the consequence of the cobalamin deficiency, as it is known that a cobalamin deficiency can affect the assimilation of cobalamin, even in presence of exogenous intrinsic factor.