The reported incidence of pregnancy-related carpal tunnel syndrome (PRCTS) ranges from 0.8% to 70%, and little is known of its natural history. We systematically reviewed the reported incidence of PRCTS and evaluated its natural history. We identified 214 studies that fulfilled our selection criteria. Six publications fulfilled the inclusion criteria. Five fulfilled the incidence criteria, 3 fulfilled the natural history criteria, and 2 of the 6 publications satisfied both. The reported incidence of neurophysiologically confirmed PRCTS ranged from 7% to 43%, whereas the incidence of clinically diagnosed PRCTS ranged from 31% to 62%. Symptoms persisted in more than 50% of the patients after 1 year and in about 30% after 3 years. Our review suggests that variations in the reported incidence of PRCTS largely depend on the methods used to detect this syndrome. Our data also suggest that symptoms persist in a substantial number of patients 1 or more years after delivery.