The plexiform fibrohistiocytic tumor (PFHT) is an infrequent soft-tissue neoplasm with uncertain biological behavior. We report a rare congenital PFHT case in a 4-year-old boy, treated with wide excision and skin grafting. After a 52-month follow-up, no recurrence, regional or distant metastases were documented. A literature review on the management of PFHTs is reported.