Familial hyperparathyroidism includes the diagnoses of multiple endocrine neoplasia type 1, type 2A, and familial isolated primary hyperparathyroidism. Familial isolated primary hyperparathyroidism is a rare, distinct form of familial primary hyperparathyroidism, mainly due to four-gland hyperplasia or single-gland adenoma. We describe our success in treating a 24-year-old woman with familial isolated primary hyperparathyroidism with resection of double adenoma, using the guide of intraoperative parathyroid hormone (PTH) monitoring. Familial isolated primary hyperparathyroidism usually presents with four-gland hyperplasia or single-gland adenoma. However, double adenoma should be considered in the differential diagnosis. Using intraoperative parathyroid hormone levels and minimal-access surgery in familial isolated primary hyperparathyroidism may be promising.