Three cases of parathyroid neoplasms in familial hyperparathyroidism are reported. There were not associated with other endocrine glands or parathyroid glands lesions. Parathyroid gland carcinoma was diagnosed in 2 cases. Review of 7 cases of familial parathyroid carcinoma, diagnosed in 5 different families and issued from the literature, indicates that it can be associated to multiple endocrine neoplasia or other parathyroid lesions. The familial parathyroid carcinoma entity needs further studies before to be fully recognised.