Introduction:We report an adult male with the sole condition of recurrent nephrolithiasis, with no other associated symptoms nor family history, who was later diagnosed with Primary Hyperparathyroidism due to a giant right superior parathyroid adenoma. To the best of our knowledge, this is one of the few well-documented cases of this rare presentation in a male patient of the Dominican Republic. This case should affect the practice of physicians who interact with primary hyperparathyroidism and are involved in their management.Case presentation: A 55-year-old hispanic man who was diagnosed and treated for the last 8 years for recurrent calcium nephrolithiasis with no other associated symptoms. Etiology for his condition remained unknown to the patient until he visited a tertiary care level hospital for routine check-up, where he was referred to endocrinology due to incidental biochemical findings of elevated PTH levels (718.10 pg/ml), hypercalcemia (13.27 mg/dl), elevated ALP (386.90 U/L), 24 hr urinary calcium of 483.04 mg/24 hr, and hypophosphatemia (2.03 mg/dl). Other symptoms common to PHPT such as abdominal pain, fatigue, weakness, psychiatric disturbances such as depression, or numbness of the skin were denied by the patient. Family history was negative for thyroid, parathyroid or kidney disease. The patient works frequently outdoors and is constantly exposed to the sun. The patient underwent a standard Tc99m-sestamibi scan that revealed heterogenous delayed persistent tracer localization in the right upper parathyroid region. An ultrasound of the neck was performed which revealed a right superior mass of the parathyroid gland measuring 4.00 × 2.48 × 2.02 cm (700% increase in size) and weighting 10.6 gm. Conclusion: Giant parathyroid adenomas are rarely encountered among patients with primary hyperparathyroidism and are known to present with distinct clinical and biochemical features that differ from typical parathyroid adenomas. To the best of our knowledge, this case report describes the first case reported in the Dominican Republic of a patient with Giant Parathyroid Adenoma (GPTA). This case report also reviews the published literature to report on the clinical characteristics and typical presentation of GPTA as well as diagnosis and treatment.