Hypothenar hammer syndrome (HHS) is a rare vascular disease caused by repetitive trauma to the hypothenar eminence, leading to thrombi and sometimes aneurysm development in the ulnar artery. This case report presents an atypical presentation of HHS with pathology extending distally into the digital arteries, complicated by an unclear history and unique genetic contributions. The patient, a 58-year-old male with a distant history of jackhammer work, presented with progressive pain in his fourth and fifth digits. CT angiography revealed patchy vascular lesions in the palmar arch. He developed another thrombi despite being treated initially with heparin. Genetic analysis revealed that he was heterozygous for Factor V Leiden with decreased protein C levels. Following surgical intervention, he recovered well. The author report a case of HHS in which medical management was superior to surgical treatment due to combined occupational and genetic predisposition for vascular pathology with findings extending beyond the ulnar artery.