Abstract Leiomyomatosis peritonealis disseminata (LPD) is a rare gynecologic disorder characterized by the presence of multiple smooth muscle nodules dispersed across the peritoneal surface. While its precise cause remains unknown, studies indicate a role for hormonal, genetic, and molecular influences in disease progression. Advances in imaging, particularly magnetic resonance imaging (MRI) and computed tomography (CT), have enhanced diagnostic precision by differentiating LPD from malignancies, while immunohistochemical markers like smooth muscle actin (SMA), desmin, and estrogen/progesterone receptors (ER/PR) offer valuable pathologic insights. Management strategies include hormonal therapy (e.g., gonadotropin‐releasing hormone [GnRH] agonists) and surgical intervention, with treatment plans tailored to disease severity and patient‐specific factors. Emerging research suggests the potential role of targeted molecular therapies in managing refractory cases. Despite these advances, LPD remains a complex condition, and further research is needed to clarify its pathogenesis, identify reliable biomarkers, and establish standardized treatment protocols. This review synthesizes current knowledge on LPD to enhance clinical awareness and guide future research on its pathogenesis, biomarkers, and treatment strategies.