Objective: The concomitant presence of pituitary adenoma (PA) with Rathke's cleft cyst (RCC) is rare, and most of the literature published is case reports. RCC's clinicopathological features have not been well described. Methods: We retrospectively reviewed the data of 15 patients with PA associated with RCC. We also provide a systematic review of the literature. Results: The patients included males and seven females. The transsphenoidal approach was adopted for all patients except one, who underwent right frontal craniotomy. Complete resection was achieved in nine cases, with grossly complete resection in five and partial removal in one. Of the PA subtypes available, five patients had nonfunctional pituitary adenomas, three had multiple-hormone secreting PAs, three had prolactin-producing adenomas, and one had a growth hormone-secreting adenoma. All of the patients' symptoms and abnormal endocrine test results resolved or were relieved after surgery. The mean follow-up time was 27.616.6 months. All of the patients recovered well and had no signs of recurrence during their follow-up, except for one patient. Conclusions: A cyst-like signal indicated on images or cystic materials encountered during operation in a patient with PA suggest the possibility of PA associated with RCC. Transsphenoidal surgery is the preferred approach and can resolve or relieve patients' symptoms to a great extent. Histologically, nonfunctional PA and prolactinoma are the most common types of PA coexisting with RCC; the mechanisms of PA associated with RCC need to be investigated further.