BACKGROUND: Acromegaly is an endocrine disorder characterized by excessive growth hormone (GH) production, causing abnormal bone and tissue enlargement. Typically, it presents with distinct physical changes, but some cases may lack noticeable features and still have internal abnormalities like aortic root dilation. Though rare, a family history can raise suspicion. CASE PRESENTATION: This report presents a case of a 23-year-old male with no significant medical history, who presented with severe dyspnea after a chest infection. He lacked distinct physical features of genetic or endocrinal disorders, but his uncle had acromegaly. Echocardiography revealed left ventricular hypertrophy and massive aortic root dilation. Elevated insulin-like growth factor 1 (IGF-1) confirmed the diagnosis of acromegaly. The patient underwent a Bentall procedure, and his acromegaly was managed with octreotide and regular follow-ups. CONCLUSIONS: The report emphasizes the potential genetic link, and the possibility of massive aortic root dilation associated with acromegaly.