Congenital infiltrating lipomatosis of the face comprises a subgroup of lipomatous tumors. While rare, it remains a definite clinical entity. Its etiology is unknown. The tumor is congenital in origin and occurs in infancy or early childhood. It is poorly enveloped and characterized by diffuse infiltration of mature adipose tissue over normal muscle fibers, rapid growth, associated osseous hyperplasia, and a high recurrence rate after surgical intervention. Due to its diffuse infiltration and involvement of important facial structures, complete surgical excision is often impossible. A total of 14 cases of congenital infiltrating lipomatosis of the face was found in a literature review. Herein we present an additional case who had both diffuse infiltrating lipomatosis and a well-encapsulated lipoma on her left cheek.