Primitive myxoid mesenchymal tumour of infancy (PMMTI) a rare and aggressive soft tissue tumour driven by alteration in the BCOR gene. Localised disease may be cured by surgical resection but metastatic disease often displays suboptimal chemotherapy response. Here we report the course of a patient with widely metastatic PMMTI, where durable disease control was achieved with the combination of apatinib, a VEGFR inhibitor, and chemotherapy with irinotecan/temozolomide.