Abstract: Objective To report the diagnosis and treatment of 1 patient with lipofibromatosis. Methods Clinical symptoms,imaging and pathological features of an adult female patient with lipofibromatosis were discussed with its relevant literature reviewed. Results The tumor was completely excised with a good outcome and its diagnosis was established according to the histological examination. Conclusion Lipofibromatosis is an extremely rare congenital disease with a high morbidity,which is usually found at the distal extremities and characterized by no clear margin,slow growth and painlessness.Its treatment should be individualized.