Germinal heterozygous mutations in the serine/threonine kinase 11 (STK11) gene were first identified as the causal mutation of the Peutz-Jeghers Syndrome, an autosomal dominant condition characterized by multiple hamartomatous polyps in the gastrointestinal tract and an increased cancer risk [1]. The gene discussed is STK11; the disease is Peutz-Jeghers syndrome.