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BACKGROUND: Leiomyomatosis peritonealis disseminata (LPD) is a rare condition characterized by formation of multiple leiomyomas in the abdominal and pelvic peritoneum. Malignant transformation of LPD is an extremely rare occurrence. We hereby report a case of a middle-aged female who was subsequently diagnosed with leiomyomatosis peritonealis disseminata with a few of the lesions showing malignant transformation. CASE REPORT: A forty-year-old female with a history of hysterectomy presenting with vague abdominal pain was completely evaluated with ultrasound (US), computed tomography (CT) and magnetic resonance imaging (MRI) and was subsequently diagnosed with leiomyomatosis peritonealis disseminata. The imaging findings of leiomyomatosis peritonealis disseminata were correlated with and confirmed by a histopathologic examination of the excision biopsy specimen. CONCLUSIONS: The imaging findings were found to be specific for leiomyomatosis peritonealis disseminata with a malignant transformation.