Livedoid vasculopathy (LV) is characterized by localized painful ulcers, livedo reticularis and atrophie blanche. LV is considered as an occlusive vasculopathy due to a hypercoagulable state. Hyperhomocysteinemia is a prothrombotic condition, which has as yet received little attention in this context. We report a 49-year-old woman with livedoid vasculopathy. The patient presented with an elevated homocysteine level caused by renal insufficiency, vitamin-B6 deficiency and reduced vitamin-B12 concentration. We treated her with low-molecular heparin and pentoxifylline. Folic acid, vitamin B6 and B12 were substituted for therapy of hyperhomocysteinemia. Substitution therapy led to a reduction of homocysteine levels. Under the complex therapy the ulcers healed without recurrence within 16 months. Thrombophilia is of pathogenetic importance in LV. Hyperhomocysteinemia may be another cause of hypercoagulability. Substitution of folic acid, vitamins B12 and B6 (cofactors of homocysteine metabolism) is an effective treatment of hyperhomocysteinemia and thus hypercoagulability can be causally treated.