Infantile fibromatosis is characterized by proliferation of fibrous soft tissue with a potential of invading the adjacent structures but lacks the ability to metastasize, thus making it a fairly benign lesion with borderline characteristics. The pathology resembles sarcomatous growth, therefore making it difficult for the clinician to correctly diagnose. There are two variants of extra-abdominal desmoid juvenile and adult variant depending upon the age group it predominately involves. Fibromatosis is comparatively a rare tumour with unpredictable growth and varying local recurrence rates. The mass usually grows slowly, rapid growth and recurrences being mostly associated with the juvenile forms. The disease may present as single or multifocal lesion with widespread distribution, thus requiring whole body scans to identify any insidious growth elsewhere. Here, we report a case of recurrent juvenile/infantile fibromatosis in a 2-year-old child, conservatively managed without any growth disturbance and signs of recurrence., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 National Journal of Maxillofacial Surgery.)