The comprehensive analysis of TPM3 gene fusions presented in this review underscores their significant role as oncogenic drivers across a diverse spectrum of malignancies including inflammatory myofibroblastic tumors, anaplastic large cell lymphoma, renal cell carcinoma, colorectal cancer, thyroid carcinoma, non‐small cell lung cancer, soft tissue sarcomas, and glioma. This evidence concerns the gene TPM3 and hereditary clear cell renal cell carcinoma.