Acromegaly can be part of a syndromic disease occurring concomitantly with other endocrine tumors, such as in MEN1, MEN4, Carney complex, McCune-Albright and SDHx-related pituitary adenomas (Table 1) or presents as part of familiar isolated pituitary adenoma (FIPA) in aryl hydrocarbon receptor interacting protein (AIP) or GPR101 (G-protein coupled receptor 101) mutation positive and negative cases [4, 5]. The gene discussed is GPR101; the disease is acromegaly.