Six personal cases of mucocele of the sphenoidal sinus are reported, and 124 cases from the literature are reviewed. Sphenoidal sinus mucocele is a benign and rare lesion which has long been unrecognized. Clinical features include fronto-orbital pain, oculomotor palsies, loss of visual acuity, exophthalmos and anosmia. Our series is of particular interest since four of our six patients presented with endocrine disorders. Recent advances in neuroradiological methods should rapidly lead to the correct diagnosis. Treatment consists of aspiration and drainage of the mucocele via a trans-sphenoidal approach. The results are good, and ophthalmoplegia usually subsides. In our experience, endocrine disorders do not always respond to treatment. The prognosis of sphenoidal sinus mucocele depends on the preoperative duration of the loss of visual acuity.