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We present the case of a 72-year-old woman who presented with, to our knowledge, the largest reported Sister Mary Joseph lesion in the literature. Often associated with a poor prognosis, the patient went on to have a wide local excision of the lesion and has made a full recovery 2 years after the nodule initially developed. Histological examination confirmed the presence of underlying endometrial cancer and the patient subsequently underwent a total hysterectomy and bilateral salpingo-oopherectomy.