Epithelioid malignant peripheral nerve sheath tumor (EMPNST) known as "epithelioid malignant schwannoma", described for the first time in 1954. It is an entity that has the same clinical features as MPNST but whose cytological and histological characters are different. It is characterized by an epithelioid cytomorphology, usually diffuse S100 immunoreactivity and some cases come from preexisting schwannomas. Clinically, it presents as a painful or asymptomatic mass, whether or not it is located on a nerve path. Imaging plays an essential role in preoperative diagnosis and assessment, but histology remains the key examination for confirmation of the diagnosis. Treatment is based on surgery, radiation therapy and chemotherapy. His prognosis is grim. The objective of this work is to underline the histopathological aspects of this very rare entity, in particular in children, by reporting an observation of a 21 month old infant.