This rare entity is characterized by multiple nodular lesions largely composed of collagen-forming spindle cells and involving the subcutis, skeletal muscle, bone and viscera of newborns and infants that occurs in either a solitary or multicentric form. The clinical course of a case is presented. The patient is a 2-year-old male who was brought to our hospital because of a large cervical mass. On physical examination a firm, subcutaneous, immobile painless mass measuring 4.5x6 cm was palpated at right side of neck. After the first admission, 13 complete surgical resections were performed. This patient is the eleventh familial case in the literature. The patient died 10 years after the first surgery.