Mutations of theBRCA1/2genes have been described in association with a number of malignancies including cancers of the breast, ovary, prostate and stomach, but have never been described in relation to gastrointestinal stromal tumours (GIST). We describe a patient with aBRCA28642del3insC mutation who developed prostate cancer, breast cancer and GIST. GIST has been shown to be associated with a number of malignancies, including some of the commonBRCA1/2-related cancers, but it has never been associated withBRCA1/2gene mutations. This report highlights the potential association betweenBRCA1/2mutations and GIST, and aims to raise awareness for further genetic screening in GIST patients.