During an antenatal echography, we observed a here to unreported fetal tumour which was diagnosed after birth as aggressive fibromatosis. The infant was treated with chemotherapy and, at the age of 27 months, still had a sclerous mass on the trunk which cause major scoliosis. Fibromatosis is a locally malignant fibrous tumour. During the unpredictable clinical course, recurrence after treatment as well as spontaneous regression is observed. Therapeutic management decisions (surgery, radiotherapy, chemotherapy) must be made on a case by case basis. Hormone factors and inappropriate oncogene expression are pathogenic factor.
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