Although hereditary PGL/PCC is a fairly distinct entity, several of our patients report family history of other carcinomas that are unrelated to PGL/PCC, including breast, colorectal, prostate, and leukaemia, suggesting differential diagnoses such as BRCA1/2 hereditary breast cancer syndrome, hereditary colorectal cancer syndrome, and Li Fraumeni syndrome. The gene discussed is BRCA1; the disease is carcinoma.