Soft tissue sarcoma, especially synovial sarcoma, is extremely rare in infancy, with only a few cases reported worldwide. Due to the particularly small size of the head and neck region in infants, with important anatomical structures in close vicinity to one another, surgical resection is very challenging, if the goal is to ensure total disease elimination as well as maintaining function and good cosmetic outcome postoperatively. In the case reported here, following diagnosis, the child was given three cycles of neoadjuvant chemotherapy followed by infratemporal tumour resection via Bramley–Al-Kayat incision incorporated with a modified Blair incision. Postoperatively, the child recovered well, with no recurrence after three months. This case highlights the important role of a multidisciplinary approach in the diagnosis and managing such rare cases. We also discuss the clinical presentation, imaging features, histopathological features, treatment regime, and prognosis of synovial sarcoma of the infratemporal fossa in children, based on cases reported in the literature.