Venous thromboembolism (VTE) with its two components, deep vein thrombosis (DVT) and pulmonary embolism (PE), is a common disease with an incidence of 0,75‑2,69/1000. Deep vein thrombosis is localized in unusual sites in about 10 %, and rare DVT's have specific symptoms and risk factors. Uncommon DVT's are often related to local factors, inflammation, cancer, hematologic diseases and thrombophilia. Their diagnostic workup is less straightforward than in suspected VTE of lower limbs and PE, and rare DVT's are often unexpected findings of imaging studies. By extrapolating evidence of lower limbs VTE, most rare DVT are treated by anticoagulants, except retinal vein thrombosis. However, evidence for the type and duration of anticoagulation is limited.