Lipofibroma of the peripheral nerve or its branches is a rare benign tumor and its pre-operative diagnosis is difficult. We reported one patient with lipofibroma of the median nerve. The patient was a nineteen-year-old boy who noticed a soft mass in his left hand thirteen years ago. The mass increased in size slowly. He was asymptomatic, the tumor mass was palpable, and there was no loss of function. An exploratory operation revealed a fusiform enlargement of the median nerve extending from the distal palmar crease to the middle finger. Epineurotomy was performed. The pathological diagnosis was Lipofibroma.