Pulmonary lymphangitic sarcomatosis (PLS) is not much recognized clinically although it shows similar pathological patterns and diagnostic features to pulmonary lymphangitic carcinomatosis (PLC). We report a case with hand angiosarcoma whose chest X-ray findings revealed a diffuse interstitial pattern consistent with lymphangitic spreading. The final diagnosis was made by open lung biopsy. The clinical, diagnostic and pathological features of this disease process are reviewed.