We describe an unusual presentation of toe ulceration in a 39-year-old otherwise healthy man. The left fifth toe was painful for several months with ulceration at the tip. The other toes appeared cyanotic and discolored. Foot pulses were normal. Laboratory testing revealed the platelet count at 1208 thousand/microliter, with hemoglobin 19.2g/dl and hematocrit 57%. The erythropoietin level was found to be markedly decreased at 1 mU/ml. The patient was given the diagnosis of polycythemia vera with iron deficiency. Phlebotomy was performed and aspirin and cytoreductive therapy with hydroxyurea was prescribed with resolution of the toe ulceration.