Knowledge of a persistent median artery, defined as an anatomical vascular variation, is of clinical importance because it can be an unusual etiology of carpal tunnel syndrome. This report describes the case of a 42-year-old female patient with acute pain and paresthesia involving the volar aspect of the first, second, and third fingers of the left hand. The clinical manifestations pointed to compressive neuropathy of the median nerve, and diagnostic imaging showed anatomical vascular variation. Details were collected through interviews with the patient, and photographic records of imaging exams were thoroughly analyzed. This case study shows the importance of the identification and knowledge about anatomical variations considering that under specific pathological conditions, they can be a cause of clinical syndromes. Such knowledge is also important in the field of surgery for the prevention of iatrogenic injuries.