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A rare case of growing teratoma syndrome (GTS) of the ovary is presented, with a review of the literature. A 36-year-old woman had 6 months’ duration of back pain and abdominal fullness. She had undergone surgery elsewhere (right salpingo-oophorectomy) followed by chemotherapy for a histopathologically diagnosed immature teratoma of the right ovary 6 years previously. Imaging showed evidence of extensive peritoneal cystic metastases indenting the liver and in the pelvis and right suprarenal region. As tumour markers were within the normal range, a diagnosis of GTS was made. The patient underwent explorative laparotomy with total hysterectomy and left salpingo-oophorectomy, and debulking of peritoneal deposits. Histopathological examination identified mature cystic teratoma. The available literature on this condition, with definitions, understanding of the pathophysiology and prognosis, is reviewed.