Tatton-Brown-Rahman syndrome (TBRS) is a rare overgrowth syndrome first described in 2014.1 This report describes a 17-year-old male with TBRS who presented with primary hyperparathyroidism (PH) and was found to have sestamibi positive imaging. The patient underwent a bilateral neck exploration where an ectopic parathyroid gland was localized and removed. The patient had no surgical complications, and his follow up visit showed normalization of parathyroid hormone and calcium levels. This report represents the importance of obtaining multiple imaging modalities during preoperative preparation for a parathyroidectomy in patients with TBRS. It also demonstrates the need for further publications of anatomic anomalies associated with PH in patients with TBRS in the future.