Neonatal primary hyperparathyroidism is a rare entity characterized by marked hypercalcemia, diffuse parathyroid hyperplasia, and skeletal demineralization. It is often lethal unless total parathyroidectomy is performed. Long-term outcome of treated patients is poorly documented. We report the clinical and radiographic outcome of this disease in a 7-year-old boy who underwent a total parathyroidectomy and autotransplantation of a fragment of one parathyroid gland to his thigh in the neonatal period. This paper demonstrates the importance of prompt diagnosis and management in neonatal hyperparathyroidism and the role of various imaging modalities in its diagnosis and follow-up.