This article provides a review of pathology, clinical features, imaging diagnosis and treatment of lipomatosis of nerve (LN), aiming to improve the comprehensive understanding of the disease and achieve early diagnosis and treatment. By searching for the relevant literature, we systematically summarized the pathology, clinical features, imaging diagnosis and treatment of LN in peripheral nerve. Lipomatosis of nerve is a rare benign tumor-like lesion in the peripheral nerve. LN can present primarily as painless neoplasm, macrodactyly, or neurological dysfunction. Magnetic resonance imaging and ultrasonography are commonly used imaging methods before operation, which can characterize and locate the scope of the disease. There are few ultrasonographic studies on LN of peripheral nerve, mostly in the form of case report or small sample analysis. Treatment of lipomatosis of nerve varies with clinical symptoms and lacks consensus. Although LN is benign lesion, it can be invasive, progressive, and recurrent. It is very important to understand the problems of neurological dysfunction caused by LN. More studies are needed to provide more detailed imaging information for clinicians and to achieve the effective treatment.