Gastrointestinal stromal tumor (GIST) and paraganglioma are uncommon tumors; mostly occur in a sporadic and isolated form. They are occasionally components of 3 clinical syndromes, familial paraganglioma and gastric GIST, neurofibromatosis type 1 and Carney triad. The latter does not show genetic mutations and affects young women predominantly. This article reports the occurrence of GIST and retroperitoneal paraganglioma in young Saudi woman aged 22 years. The initial clinical presentation was acute severe gastric bleeding. Gastric mass with retroperitoneal mass was discovered during surgical exploration. Histological and immunohistochemical study of both masses showed features of GIST and paraganglioma. The combination of GIST and retroperitoneal paraganglioma in the reported case may be incomplete expression of Carney triad or be coincidental occurrence.