A 20 year-old male had presented at the age of 11 years with abdominal pain and anaemia, having previously been well, and was found to have a haemorrhagic retroperitoneal tumour requiring prompt excision. The tumour was a hormonally-inactive paraganglioma; a germline SDHB mutation was found. His father, paternal grandfather, four paternal aunts and uncles and two cousins were also found to carry the mutation, though our proband was the only one with an identified paraganglioma. He had recurrent episodes of adhesion-related small bowel obstruction during his teens. During a laparoscopic adhesiolysis at age 19 he was observed to have two solid liver lesions and two large gastrointestinal lesions. He was normotensive with normal range plasma metanephrines, but a Ga68-DOTATATE PET scan showed metastatic paraganglioma to the lung, retroperitoneum, spine, and frontal bone. The co-registered CT scan showed three low density gastrointestinal tract lesions and several liver lesions. On FDG-PET scanning the bowel and liver lesions showed uptake consistent with metastatic gastrointestinal stromal tumour (GIST) and metabolically active metastatic paraganglioma. With the primary abdominal mass measuring 90mm, along with liver masses measuring up to 28mm, a decision was made in consultation with medical oncology and the hepatopancreatobiliary surgeons to proceed with a distal gastrectomy and liver wedge resection. Histopathology confirmed metastatic GIST, and in the context of metastatic paraganglioma, the diagnosis of Carney-Stratakis syndrome was made. This autosomal dominant syndrome with incomplete penetrance is characterised by paraganglioma and GIST, recognised as occurring due to germline mutations in succinate dehydrogenase gene subunits, mainly SDHB, SDHC or SDHD. Loss of function of these tumour suppressor genes predisposes to the development of this dyad. We depict the early course of this young patient with Carney-Stratakis syndrome and a description of his management in the context of what is known about this rare condition.