Background: Coronary interventions in patients of achondroplasia have been reported rarely in the medical literature. Due to short stature and kyphoscoliosis, endovascular access (Cannulation) of the coronary arteries is usually extremely difficult in such patients. Case presentation: A thirty-three years old patient, a known case of achondroplasia, presented with epigastric pain for three hours duration to a university hospital, Sudan. A trans-femoral approach for coronary angioplasty was preferred. After it has been extremely difficult to cannulate the left system at first, the cannulation has been performed successfully using 5F, JL3.5 catheter. The angiogram depicted total occlusion of the proximal right coronary artery which was found to be originating from the left coronary sinus of the aorta. Successful trans-femoral coronary angioplasty has been performed with stent placement, and no complications encountered. During her last follow up, one year after the procedure, she appeared to be free of symptoms and with no further ischemic attacks or procedure-related complications. Conclusions: It is the fifth case in the medical literature of coronary angioplasty in achondroplasia patients. However, this is the first reported case of successful coronary angioplasty in achondroplasia patient in whom the occluded artery is an anomalous coronary artery. Literature review, description of the achondroplasia, development of the coronary arteries and the hypothesized theory for the anomaly have been described in this case report. The PCI performed has also been clearly and comprehensively described.