In the present case, NGS revealed a missense mutation in STK11 on chromosome 19 (c.1062C > G) and in STK11IP on chromosome 2 (c.2G > T, c.1687G > A, c.2255C > T), indicating that STK11 variants in PJS may contribute to LEGH pathogenesis. The gene discussed is STK11IP; the disease is Peutz-Jeghers syndrome.