Juvenile hyaline fibromatosis (JHF) is rare disease of autosomal recessive inheritance.A 14-year old boy, born to consanguineous parents was admitted because he suffered from multiple tumors since the age of 5 years. These tumors were mobile, painful varying in size, located mainly on the head, the back, and extremities; they were associated with gingival overgrowth. Bone X-rays showed osteolytic lesions. The cognitive development was normal. Biopsy of tumors showed cords of spindle-shaped cells embedded in homogeneous eosinophilic matrix. The child was progressively disabled due to articular changes. Two of his brothers presented the same features.This new case of JHF confirms the severity of prognosis of such a fibromatosis presently considered as a hereditary disorder of collagen metabolism.