Background: Leiomyomatosis Peritonealis Disseminata (LPD) is characterized by multiple smooth muscle intra-abdominal and pelvic nodules that may recur after complete resection. Predominantly described in females of childbearing age, LPD has only isolated case reports indicating its malignant potential, and a comprehensive collation of cases is lacking. This manuscript presents the first comprehensive collation of published data on LPD to provide insights into its management. Additionally, we present a case of a single patient's progression from benign to malignancy over two decades, highlighting the need for long-term follow up. Methods: We present a case of LPD's malignant transformation over two decades. Initially diagnosed with a uterine atypical smooth muscle tumor, the patient later developed frank malignancy, resulting in liver and lung metastases almost 23 years later, as confirmed by our included imaging. Additionally, we compiled an Excel database of all 213 available cases in English literature, documenting hormone receptor status, age, tumor size, follow-up time, and surgical interventions. Patients were categorized into G1 (reportedly benign), G2 (malignant at presentation), and G3 (malignant transformation). Results: Compared to G1, patients in G2 were more likely to be symptomatic (73% vs. 88%), larger in size (4.1 cm vs. 8.4 cm), and older in age (38 vs. 44 years). The average age in G1, G2, and G3 was 38.5, 44.3, and 37.5 years, respectively, while disease-specific survival was 100%, 71%, and 40%. The mean number of surgeries performed in G1, G2, and G3 was 1.6, 1.8, and 3.8, respectively. Hormone receptors were found in 24.4% of cases. The mean reported follow-up time in G1, G2, and G3 was 44.9, 13.1, and 70.5 months, respectively. This suggests that with longer follow up, even apparently benign tumors may develop malignancy. The transformation time to malignancy in G3 was 77.8 months, which is more than the average reported follow-up in G1 (32.9 months). Conclusion: Although LPD has been considered benign, this case shows a significant lag between initial diagnosis and malignancy development. LPD is potentially malignant with a long latency period before transformation. Lifelong surveillance should be considered even for cases presumed benign. Our results suggest that loss of hormone receptor expression may indicate malignant transformation. Circulating tumor DNA (ctDNA) levels could be associated with hematogenous metastases and serve as a novel biomarker.