A phase II trial enrolling patients with alveolar soft part sarcoma (ASPS), characterized by ASPSCR1–TFE3 fusion gene and consequently with an upregulation of vascular endothelial growth factor (VEGF), explores the combination of pembrolizumab with the VEGF receptor inhibitor, axitinib. The gene discussed is VEGFA; the disease is alveolar soft part sarcoma.