Summary

A 27-year-old woman with junctional epidermolysis bullosa (EB) presented with primary amenorrhoea and massive haematometra. Considering her severe physical handicap and anticipated problems in optimising menstrual hygiene, the patient and her parents opted for hysterectomy which was performed uneventfully. The patient was recovering well postoperatively until the eighth postoperative day when she developed acute dilation of stomach and unfortunately died on day 17.