We are aware of only four previous cases of Kveim positive sarcoidosis reported in hypogammaglobulinaemic patients and we describe two further patients. Patients with hypogammaglobulinaemia and a sarcoid-like syndrome or sarcoidosis have been reviewed. Similarities between sarcoidosis and a subgroup of late onset primary hypogammaglobulinaemia have been highlighted. It is suggested that sarcoidosis in this patient population is probably commoner than was previously thought, but the association between the two conditions remains unclear.