We report a case of infrasellar craniopharyngioma in a 34 year-old woman who presented with progressive headache and diplopia. Computed tomographic and magnetic resonance images showed a heterogeneous tumor originating from the sphenoid bone with ethmoid sinus and sella turcica extension. A sublabial rhinoseptal transsphenoidal surgery was performed. Craniopharyngiomas with infrasellar development are very rare. Infrasellar craniopharyngioma is uncommon, thirty-five cases has been reported in literature. The embryology, clinical features and radiographic investigation of these tumors are discussed.