A case of juvenile hyaline fibromatosis in a 7-year-old Asian boy is presented. This autosomal recessive inherited condition has not been described in the otolaryngology literature before. We demonstrate the benefits surgical intervention, for treatment of gingival hypertrophy, can bring to the patient and outline the other features of this rare condition. The literature on juvenile hyaline fibromatosis and infantile systemic hyalinosis is also reviewed.