Testicular microlithiasis (TM) has been associated with testicular germ cell tumours (TGCTs) in adolescents and adults and with its precursor carcinoma in situ (CIS). A clear definition of TM and the need for further diagnostics and follow-up is lacking. We reviewed the literature of TM and its association with TGCT/CIS and current follow-up advises and propose a management approach based on associated risk factors for TGCT. In the literature, a wide variance of TM incidence is reported in different patient populations. A consensus concerning the malignant potential of TM has not been reached. In addition, a clear definition on TM is lacking. Although a correlation between TM and TGCT or CIS is found, precise management and follow-up schedules are absent. We suggest that all hyperechogenic foci smaller than 3 mm without shadowing should be named TM irrespective of their number. In addition, we suggest a management scheme for physicians encountering TM in daily practice. Our algorithm suggests taking a testicular biopsy in a selected patient population with at least one additional risk factor for TGCT. A long-term active follow-up schedule, including ultrasonography and physical examinations, is not indicated in the remaining patients with TM.