Pyoderma gangrenosum is a rare neutrophilic dermatosis manifesting as painful pustules or bullae, which rapidly become necrotic to produce an ulceration. It is often associated with systemic disease, such as inflammatory bowel disease, rheumatoid arthritis, leukemia, but myelofibrosis is reported rarely for its underlying disease. The most common location is the lower extremities, and the pyoderma gangrenosum also occur infrequently on the hand and face. As in our case, bilateral involvement of the hands are rarely reported. We present a case of pyoderma gangrenosum that had developed on both hands in a 66 year old man who had macrocytic,anemia. A bone marrow biopsy revealed proliferation of fibroblasts and a dense reticulin network consistent with myelofibrosis, the association of which is very rare world wide. (Kor J Dermatol 1997;35(4): 809-813)