The congenital infiltrating lipomatosis (LCI) represents a distinct, clinical and pathological aspect of fat soft-tissue neoplasia; very few cases of which have been described in literature. In 1983, Slavin and Cols described the main characteristics of LCI as:(1) non-encapsulated tumours containing mature adipocites, (2) fat infiltration in muscles and adjacent soft parts, (3) absence of malignant characteristics, (4) absence of lipoblasts, (5) presence of fibrous elements, increased number of vessels and nerves and (6) adjacent bone hypertrophy. In this article, we describe a congenital infiltrating lipomatosis of the face in a child and discuss the diagnoses and the treatment of this disease.