Background <p>Hereditary multiple osteochondromas (HMO) are benign neoplasms that predominantly affect the bones around the knee joint, proximal humerus, wrist, pelvis, and to a lesser extent, the rib. Although rib-origin osteochondromas are uncommon and often asymptomatic, they can lead to pleural, pericardial, and diaphragmatic injuries secondary to tumour related pressure. We are presenting a unique case of a vascular-related giant osteochondroma rib that is hereditary, originating from the right second, third, and fourth ribs causing compression symptoms in a young child. The child underwent wide local excision and reconstruction using polypropylene mesh that was managed successfully with no recurrence.</p> Case presentation <p>A 9-year-old male experiencing fullness and pain in the right chest for two months with a parallel medical history of swellings in first- and second-generation relatives that is hereditary in origin underwent contrast-enhanced computed tomography revealing a sessile rib osteochondroma arising from the anterior aspects of the right second to fourth ribs with an associated large cartilaginous cap. The bony growth measured 4.5 × 2.5&#xa0;cm indenting the right upper lobe while, its cartilaginous cap measured 2.5 × 4.8&#xa0;cm posterior-superiorly seen encasing the axillary artery and 3D volumetric reconstructive image revealed the proximity of the axillary and subclavian artery with the lesion. A biopsy was inconclusive with no malignant cells, so he underwent wide local excision of the tumour and reconstruction with polypropylene mesh. Follow-ups at one, three, six, and nine months were uneventful with normal thoracic curvature and no sign of recurrence.</p> Conclusion <p>This case illustrates the surgical challenges addressed and the successful outcome of a paediatric chest wall reconstruction in a growing child, utilizing advanced imaging techniques, to underscore the importance of an individualized, innovative approach in managing rare skeletal anomalies.</p>