Primary hyperparathyroidism is the third most common endocrine disease, while primary hyperparathyroidism associated with non-Hodgkin lymphoma(NHL) is extremely rare. We report a case of primary hyperparathyroidism associated with NHL. The first symptom of this patient was hypercalcemia. Hypercalcemia is the primary first clinical manifestation of primary hyperparathyroidism, while NHL may also be diagnosed by hypercalcemia. Clinically, patients with hypercalcemia as the first symptom should be alert to the coexistence of their two diseases. As primary hyperparathyroidism and NHL occur simultaneously, the relationship between the two diseases requires further study. A review of the literature regarding primary hyperparathyroidism associated with NHL was performed, focusing on clinical presentation, diagnosis, treatment, prognosis, and the connection.