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A 33-year-old male consulted us with symptoms of band-like infiltration, thickenning and subcutaneous induration occurring in the face, chest, back, shoulder and upper extremities. In the following time he developed arthralgia, fever and ocular symptoms. Laboratory tests revealed normal thyroid functions and a normal bone marrow picture, but there were immunological abnormalities such as reduced cellular immunity, decrease in C 3 (beta 1c/la) and positive antinuclear antibody. Histologically, there was an increase in fibroblasts and deposits of hyaluronic acid were revealed histochemically. A diagnosis of lichen myxedematosus was established. At present, all symptoms and signs including skin rash have disappeared following corticosteroid therapy and the patient is being followed up. So far as we have been able to trace, reports on lichen myxedematosus associated with immunological abnormalities as described here have not been found. The relationship between this pathology and immunological abnormalities are discussed.