Objective: This report aims to present an unusual case of primary lymphoma of the pituitary stalk, and provide more evidences and treatment approaches for this rare disease. Case presentation: A 66-year-old female presents as headache, dizziness, polyuria, polydipsia and visual deterioration. Brain MRI revealed isolated pituitary stalk thickening (>5 mm) without pituitary mass, but with intense and homogeneous enhancement. Serological test for HIV antibodies was negative. Water deprivation test and vasopressin test confirmed central diabetes insipidus. The patient underwent right pterional craniotomy to resect the mass. The infiltrating lymphoid cells stained virtually positive for terminal deoxynucleotidyl transferase (TdT), CD20 (B-cell marker), CD79a, LCA, CD43 and CD99; while CD3 (T-cell markers), cytokeratin (CK), epithelial membrane antigen (EMA), CD5, CD23, S-100 protein, nuclear transcription factor (NF), and chromogranin A (CgA) were negative. Proliferation index by Ki-67 was greater than 70%. A definitive diagnosis of primary B-cell lymphoblastic lymphoma was made. Conclusion: This case shows that it is a necessary to be vigilant to endocrine and metabolic manifestations in patients with malignant central nervous system (CNS) involvement. Primary CNS lymphoma (PCNSL) should be considered as a potential cause of sellar mass.