A 49 year old woman presented with leiomyomatosis peritonealis disseminata (LPD) which is a benign and very rare disease entity. This patient is particularly interesting because she presented with many atypical features. She is the second Oriental case reported, and the follow‐up period was long. The disease was symptomatic and progressive despite bilateral salpingo‐oophorectomy, hysterectomy and repeated excision of all the recurrent nodules. She had documented recurrences of LPD in three episodes at 4 years apart. There was no obvious systemic hormonal abnormality including pregnancy or oestrinizing granulosa tumour, and she had never taken any contraceptive drugs. The importance of differentiation of this condition from generalized metastatic intra‐abdominal malignancy is discussed as LPD has a very good prognosis.