An uncommon but clinically relevant subset of tumours with gastrointestinal tract origins are called gastrointestinal stromal tumours (GISTs). These tumours typically develop in the stomach and small intestine, which are connective tissues of the digestive system. However, they can also develop in other areas of the gastrointestinal tract, such as the oesophagus, colon, and rectum. GISTs are distinctive in that they are frequently identified by the presence of mutations in genes that encode receptor tyrosine kinases, such as KIT or PDGFRA. Tumours are created as a result of the unchecked growth and proliferation of cells caused by these genetic abnormalities. The diverse nature of GISTs is one of its distinguishing characteristics. Small, benign growths to aggressive, malignant tumours are all possible. GISTs can present clinically in a variety of ways, with symptoms frequently depending on the location, size, and propensity for complications of the tumour. Abdominal pain, gastrointestinal bleeding, a palpable lump, or even unrelated incidental discoveries during medical imaging are examples of common symptoms. Effective management depends on early diagnosis since it enables prompt intervention and better results. GISTs are normally diagnosed using a combination of physical examination, medical history evaluation, imaging tests (such CT scans and MRI), and tissue biopsies. The presence of particular immunohistochemical markers in the tumour tissue, such as CD117 (KIT), CD34, and DOG1, is the gold standard for validating the diagnosis of GIST. Following a diagnosis, GISTs are further divided into risk groups according to the location, size, and mitotic rate (the pace at which cells divide), which informs treatment choices. Gastrointestinal Stromal Tumours, as a group of neoplasms, are complicated and exhibit a wide range of clinical behaviours. The prognosis for patients with GISTs has considerably improved thanks to developments in our understanding of the underlying genetics and the creation of tailored medicines. In order to effectively manage GISTs and give patients the best outcomes possible, early diagnosis, risk assessment, and individualised treatment programmes are crucial. Clinical trials and ongoing research are exploring new treatments and honing our strategy for treating this difficult class of tumours.