Introduction: Acromegaly is a rare endocrine disorder of the growth hormone (GH)-insulin-like growth factor 1 (IGF1) metabolism that can affect women of fertile age. Although uncommon, pregnancies in acromegalic women can occur, with data regarding the management of these cases being very limited, mostly consisting of case reports. Case Presentation: We present the case of an acromegalic woman, first diagnosed at the age of 22, after the surgical resection of a pituitary mass. Throughout the evolution, she received conventional radiotherapy and has been treated with somatostatin receptor ligands (SRLs), dopamine agonists (DAs), and GH-blockers. At the age of 37, the patient decided to become pregnant while she was on Pegvisomant and DA therapy. The treatment was stopped, and the patient became pregnant at the age of 38. Tumor size and IGF-1 values have remained stable throughout the pregnancy, and no complications occurred. A healthy child with normal birth weight was delivered on term through Cesarean section. Conclusions: Managing pregnant women with acromegaly is challenging because of the little available data regarding the safety of medical treatment and a high interindividual variability of GH-IGF-1 evolution during this period. The particularity of our case was a patient with a long history of acromegaly, who had an uneventful pregnancy despite stopping all medical treatment.