The association of testicular microlithiasis with testicular tumor and the management of incidentally detected testicular microlithiasis have generated a great deal of interest. We review the current literature on testicular microlithiasis with regard to its association with testicular tumor. This association seems complex. The available data suggest that men with incidental findings of testicular microlithiasis but who have otherwise normal testes are at low risk of developing testicular cancer. The only follow-up recommended is regular testicular self-examination. Testicular microlithiasis is, however, associated with a high risk of developing testicular malignancy in men with subfertility, history of contralateral testicular tumor or history of cryptorchidism. Regular testicular self-examination is recommended for follow-up of high-risk patients, but the role of surveillance with serial ultrasonography and measurement of tumor markers is still not clear.