We describe the case of a 60-year-old woman with CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) associated pulmonary hypertension undergoing transradial coronary angiography. The day after the procedure, the patient complained of severe symptoms and signs of acute hand ischemia. Urgent right upper extremity angiography showed the lack of ulnar palmar arch and a severe narrowed radial artery with endoluminal filling defect. The patient was successfully treated with manual thromboaspiration leading to a complete flow restoration and symptom relief. This case shows that radial occlusion, one of the most common and usually asymptomatic complications following transradial cardiac catheterization, may cause severe hand ischemia in patients with small-vessel inflammatory disease.