HighlightsPrimary umbilical endometriosis is rare and the presence of an underlying hernia makes it a diagnostic challenge.Only 7 cases have been reported in the literature.Our patient underwent an en-bloc resection of the hernia sac, endometrioma and umbilicus with reconstruction. There is no disease recurrence at 6 months.MRI is the preoperative imaging modality of choice and there is a limited role for preoperative FNA and medical management.