Several genetic syndromes have been associated with smooth muscle tumors, including FH Tumor Predisposition Syndrome (Hereditary Leiomyomatosis and Renal Cell Cancer [HLRCC]), Alport syndrome, PTEN Hamartoma Tumor Syndrome, MEN1, CDC73-Related Disorders/Hyperparathyroidism-jaw tumor syndrome, and the Birt-Hogg-Dubé Syndrome [See GeneReviews® (33)]. The gene discussed is MEN1; the disease is PTEN hamartoma tumor syndrome.