Multiple symmetric lipomatosis is a rare disease characterized by progressive growth of subcutaneous fat masses which are located symmetrically at neck, shoulders, chest, abdomen and groin. Recent surveys revealed a high incidence of combined somatic and autonomic neuropathy. The exact cause of the disease is not known. We have experienced one case of multiple symmetric lipomatosis with mediastinal involvement with symptomatic compression of trachea. The patient was a 55-year-male, complaining of dyspnea and slowly enlarging multiple symmetric masses at the neck, shoulders, chest, abdomen, flank and groin over a period of 10 years. He had a habit of excessive alcohol intake for many years. The fatty masses in the neck and the upper mediastinum including peritracheal region were excised through transverse cervical incision. But, because of the incomplete excision of peritracheal fatty tissue, we performed reoperation for the relief of residual tracheal compression at the 15th postoperative day. Two days later emergent tracheostomy was performed due to postoperative pneumomediastinum and subcutaneous emphysema. He could discharge with permanant tracheostomy.