Infantile fibrosarcoma (IFS) is an extremely rare locally aggressive soft tissue tumour of childhood. Primary therapy involves complete surgical resection with or without chemotherapy. However complete surgical resection might not be feasible in all cases and so requires other modalities for further management. We report the case of a male infant from Bangladesh with a locally advanced IFS of the leg which was partially resected. The patient received adjuvant chemotherapy which was complicated by the development of chemotherapy-related veno-occlusive disease and had to be discontinued. Thereafter he was referred to our dedicated sarcoma oncology clinic in India for further management. The parents of the child refused amputation of the limb. The tumour tested positive for NTRK3-ETV6 gene fusion and after discussion in multidisciplinary clinic, targeted therapy using oral NTRK inhibitor larotrectinib was started. The patient had complete response at the end of 8 months of treatment with larotrectinib. This is the first report from the Indian subcontinent and we encourage that these children should be referred to specialist clinics for appropriate multidisciplinary management for best outcomes.