Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract. It can be benign or malignant in behavior. Stomach, being the most common site (70%) for GISTs, is followed by the small intestine (20%). Anorectal, colonic, and esophageal GISTs are uncommon, whereas extraintestinal GISTs are extremely rare. The presenting symptoms are highly dependent on tumor size and location, although a large number of GISTs are asymptomatic. The purpose of this article is to highlight the unusual characteristics of GISTs illustrated by PET/CT and CT imaging. These characteristics are organized into (1) unusual tumor location, (2) unusual clinical presentation, and (3) unusual sites of metastasis. Knowing the uncommon features of GISTs is important, as they are more often seen nowadays with the increasingly important role of PET/CT and CT in GIST management; and these tumors are associated with a poorer prognosis and unwanted delay in diagnosis is avoidable. With the availability of effective treatment by imatinib mesylate, a prompt and early diagnosis is essential for disease control. All GISTs illustrated in this article are pathologically proven.