| Summary: | Abstract Background A Rathke’s cleft cyst is a benign intrasellar cystic lesion, with suprasellar extension reported in a few cases. An entirely suprasellar Rathke’s cleft cyst without any intrasellar component, though previously reported, is rare. Case presentation We report a case of an entirely suprasellar Rathke’s cyst in a 25-year-old female presenting with headache and visual complaints. The patient was treated surgically by drainage of the cyst and removal of the cyst wall by transcranial approach. Histopathology and immunohistochemistry confirmed the diagnosis of Rathke’s cyst. We review the relevant literature and discuss its neuroembryologic pathogenesis, pathology and the factors influencing the recurrence of the cyst. Conclusions To the best of our knowledge, only 62 cases of an entirely suprasellar Rathke’s cyst have been reported till date.
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