Primitive myxoid mesenchymal tumor of infancy (PMMTI) is rare with aggressive clinical course and a new category of pediatric fibroblastic–myofibroblastic tumor. It previously may have been included under the diagnostic categories of congenital-infantile fibrosarcoma, undifferentiated sarcoma or infantile fibromatosis. The tumors occurred on the trunk, extremities and head and neck. There are no reported case of this tumor occurring in the facial–buccal region. After surgery and regular follow up the patient is still alive until 3 years old now.

Conflict of interest: None declared.