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Gastrointestinal stromal tumor (GIST) is the most common malignant neoplasm of mesenchymal origin. GIST spans a wide clinical spectrum that ranges from tumors with essentially no metastatic potential to malignant and life-threatening spread diseases. Gain-of-function mutations in KIT or PDGFRA receptor tyrosine kinases are the crucial drivers of most GISTs, responsible for tumor initiation and evolution throughout the entire course of the disease. The introduction of tyrosine kinase inhibitors targeting these receptors has substantially improved the outcomes in this formerly chemoresistant cancer. As of today, five agents hold regulatory approval for the treatment of GIST: imatinib, sunitinib, regorafenib, ripretinib, and avapritinib. This, in turn, represents a success for a rare neoplasm. During the past two decades, GIST has become a paradigmatic model in cancer for multidisciplinary work, given the disease-specific particularities regarding tumor biology and tumor evolution. Herein, we review currently available evidence for the management of GIST. This clinical practice guideline has been developed by a multidisciplinary expert panel (oncologist, pathologist, surgeon, molecular biologist, radiologist, and representative of patients' advocacy groups) from the Spanish Group for Sarcoma Research, and it is conceived to provide, from a critical perspective, the standard approach for diagnosis, treatment, and follow-up., Competing Interests: CS has received research funding (institution) from IDRX, Karyopharm, Pfizer, Deciphera, and Bayer; consulting fees (advisory role) from CogentBio, Immunicum AB, Deciphera, and Blueprint; payment for lectures from Roche, PharmaMar, Bayer, and Blueprint; and travel grants from Gilead, PharmaMar, Pfizer, and Bayer AG. JMB has received research funding (institutional) from Adaptimmune, Amgen, AROG, Bayer, Blueprint; BMS, Celgene, Daiichi Sankyo, Deciphera, Local PI; Eisai, FORMA, GSK, IMMIX Biopharma, Karyopharm, Lilly, Lixte, NEKTAR, Novartis, Pfizer, and PharmaMar. And honoraria for advisories and lectures from Asofarma; Bayer, Eisai; Lilly; PharmaMar, and Tecnofarma. Section editor in Ther Adv Med Oncol. JALG has received research funding (institution) from Generalitat Valenciana and European Commission; consulting fees (advisory role) from AstraZeneca and Diaceutics; payment for lectures from AstraZeneca, GSK, and Astellas. XG has received research funding (institution) from AstraZeneca; consulting fees (advisory role) and lectures from Roche, Pharmamar, BMS, Pfizer, Ipsen, EusaPharma, Eisai, and Astellas. The remaining authors declare no competing interests., (© The Author(s), 2023.)