An extremely rare case of parathyroid cancer in a hemodialysis patient is presented here. A 69-year-old man was operated on for secondary hyperparathyroidism due to chronic renal failure. A locally invasive tumor of the right inferior parathyroid gland and enlargement of the other three parathyroid glands were found during bilateral neck exploration. Surgical management consisted of en bloc resection of the right inferior parathyroid gland with the right thyroid lobe and part of a sternothyroid muscle along with subtotal parathyroidectomy of the three enlarged parathyroid glands. Histological examination of the resected tumor provided evidence of a parathyroid cancer in the right inferior parathyroid gland. Nodular hyperplasia was confirmed histologically in the other three glands. With less than 20 previous cases described in the literature, parathyroid cancer is a very rare condition in hemodialysis patients.