Extraintestinal gastrointestinal stromal tumors (GISTs) are extremely rare, and adrenal GISTs are even exceptional. Only three cases have been reported in the literature thus far, the current case being the fourth. This case demonstrates the need of including extraintestinal GIST in the differential diagnosis when investigating adrenal tumors. Herein, we present a case of adrenal GIST diagnosed in a 60-year-old female patient who had a left adrenal GIST surgically removed as an adrenal tumor.