Intracranial epidermoid cysts are benign lesions of epithelial origin that most frequently present with symptoms of mass effect. Although they are often associated with a high rate of residual tumor and recurrence, maximal safe resection usually leads to good outcomes. The authors report a complete resection of an uncommon pituitary stalk epidermoid cyst with intrasellar extension using a combined suprasellar and infrasellar interpituitary, endoscopic endonasal transsphenoidal approach. The patient, a 54-year-old woman, presented with headache, visual disturbance, and diabetes insipidus. Postoperatively, she reported improvement in her visual symptoms and well-controlled diabetes insipidus using 0.1 mg of desmopressin at bedtime and normal anterior pituitary gland function. One year later, she continues to receive the same dosage of desmopressin and is also taking 50 mcg of levothyroxine daily after developing primary hypothyroidism unrelated to the surgical procedure. A combined infrasellar interpituitary and suprasellar approach to this rare location for an epidermoid cyst can lead to a safe and complete resection with good clinical outcomes.