VEGFA and cancer: The case highlights three key implications, namely: (1) validation of bevacizumab’s dual therapeutic potential against PJS-associated malignancies and cutaneous manifestations, (2) proposal of mucocutaneous pigmentation as a dynamic biomarker for treatment response monitoring, and (3) urgent need for prospective clinical trials evaluating VEGF inhibition in PJS through longitudinal surveillance of oncologic control and pigment dynamics.