The objective of this study is to discuss the myomatous erythrocytosis syndrome in a patient with a giant subserous uterine myoma. She presented with plethora and an abdominal mass. After venesection of 4 units of blood, the preoperative haematocrit value of 53.3% and haemoglobin value of 17.5 g/dL had decreased to 48.6% and 16.8 g/dL levels, respectively. After the operative extraction of the giant subserous myoma with attached uterus weighing 14.2 kg, the haematocrit and the haemoglobin values had regressed to 40.3% and 14.3 g/dL levels, respectively. The findings indicated that the giant subserous myoma was the cause of the myomatous erythrocytosis syndrome in this patient.