Malakoplakia is a rare chronic inflammatory disease that mainly affects the genitourinary system. A case of malakoplakia in the retroperitoneal space in a 53-year-old female patient with abdominal pain syndrome is presented. According to the results of computed tomography and magnetic resonance imaging of the abdominal organs and retroperitoneal space, the changes were assessed as nonspecific. Diagnostic biopsy revealed specific microscopic signs — the presence of inflammatory cell infiltration in the soft tissues of the retroperitoneal space with an abundance of CD68-positive macrophages containing PAS-positive cytoplasmic inclusions — Michaelis-Gutman bodies. Morphological examination allows differential diagnosis of malakoplakia with other pathological processes.