Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited disorder characterized by intestinal hamartomatous polyps with a distinct pattern of melanin deposition in skin most commonly perioral regions. Patients with PJS are 15-times more prone to develop GI malignancies. PJS patients are at high risk for developing pancreatico-biliary malignancies 1. There is also an association of ovarian neoplasm with PJS, most commonly granulosa cell tumor 2.
The patient had undergone GI endoscopy and biopsy and the diagnosis of PJS was confirmed on the histopathological appearance of a hamartomatous polyp.