A variety of diseases, other than the common Lev-Lenègre disease, are associated with cardiac conduction system abnormalities. These include acute processes, such as acute rheumatic fever, and other disorders, such as sarcoidosis, connective tissue disorders, neoplasms, and bacterial endocarditis with cardiac abscess formation. The purpose of the study is to raise awareness of these rare conditions. We present 10 adult patients (4 males and 6 females) with a mean age of 47 years (range: 19-69), with various rare diseases associated with heart block, who needed temporary or permanent pacemaker therapy in the past two decades. These conditions included acute rheumatic carditis, Wegener granulomatosis, cardiac involvement of metastatic breast cancer, bacterial endocarditis, sarcoidosis, S/P chest radiotherapy, and quadriplegia with syringomyelia postspinal cord injury, and adult congenital heart block. We conclude that patients with these disorders should be followed periodically, to allow for early detection and treatment of cardiac conduction disturbances, with pacemaker therapy.