The Bertolotti syndrome is a rarely recognized cause of low back pain, attributed to a congenital anomaly in the formation of the transverse processes of L5. This case report details the experience of a 22-year-old professional cyclist who transitioned from persistent low back pain to ultimately undergoing a successful surgical intervention for Type IIa Bertolotti syndrome. The initial conservative approaches, including exercise therapy and infiltrations, yielded temporary relief. Subsequent surgical intervention resulted in significant and sustained pain relief, enabling the cyclist to resume professional cycling. The Bertolotti syndrome, rarely acknowledged as a cause of low back pain, necessitates accurate identification for appropriate management. A step-up conservative therapy plan is strongly favoured before considering surgery. However, this case highlights successful surgical outcome in a professional athlete and underscores the importance of individualized management.