Multiple hereditary exostoses (MHE) is a rare skeletal disorder inherited as an autosomal dominant disorder. It is characterized by widespread multiple osteochondromas that grow near bone growth plates, leading to pain and deformities that significantly impact physical and emotional well-being and disrupt daily activities, social interactions, and psychological health, leading to considerable disability. This case report describes a 15-year-old boy with a family history of MHE who developed a large osteochondroma at his right elbow. We aim to present the surgical management of extraordinarily large-size proximal radius osteochondroma, fortunately, caused by a benign underlying condition despite typically carrying more chances of transformation into malignancy. To the best of our knowledge, it would be the largest proximal radius osteochondroma documented in the literature.