Infantile myofibromatosis of the ovary presenting with ascites

We report the first case of ovarian infantile myofibromatosis (IM) presenting with gross ascites in a 2-month-old girl. There was no evidence of recurrence of ascites or tumour 5 years after complete excision. IM has to be considered in the differential diagnosis of ovarian tumours in infants. Despite having the alarming histologic features of high cellularity, brisk mitotic activity, vascular invasion and necrosis, the tumour responded well to surgery alone without aggressive adjuvant therapy. The case is reported to expand the spectrum of 1) ovarian tumour pathology; 2) infantile myofibromatosis distribution; 3) causes of infantile ascites.