Adenoid cystic carcinoma (ACC) is a rare and aggressive malignant tumour predominantly found in the salivary glands but can also occur elsewhere. Primary ACC in the bones is particularly rare, with most reports focusing on cases of ACC in the maxilla and mandible. This report presents a case of ACC in a 47-year-old man with a primary origin in the right rib region, a previously unreported location. Its imaging manifestations and pathological features, as well as surgical treatment and follow-up, were described to increase awareness of this rare disease.