Isolated distal deep vein thromboses (IDDVT) represent up to 50 % of legs deep vein thromboses (DVT). However, since their natural history is to date unknown, the need to diagnose and treat them is a matter of debate. The diagnostic strategy based on the assessment of pre-test probability and D-dimer demonstrated a scarse efficiency for IDDVT. The choice between a proximal and a complete ultrasonographic approach should be guided by the clinical context, the local expertise and the patient characteristics. Randomized and observational studies have analyzed the need of therapy and compared different regimens of anticoagulation, with conflicting results. Systematic reviews and meta-analyses tend to support the usefulness of an anticoagulant treatment, even if the optimal dose and duration are not still defined. A careful stratification of the patient’s profile, taking into account risk factors for proximal extension, recurrence and bleeding should address the therapeutic approach, which must always be discussed with an adequately informed patient. Further studies aimed to clarify the natural history of IDDVT, and to assess safety and efficacy of lower intensity and shorter duration protocols are urgently needed.