Background Solitary sphenoid sinus pathology is uncommon. The deep position of the sphenoid sinus makes symptoms related to solitary sphenoid sinus pathology non-specific and contributes to a significant diagnosis delay. Moreover, surrounding anatomical elements cause its pathology to be potentially serious and make surgical management challenging. Methods This retrospective study includes 7 cases presented with primary benign sphenoid sinus pathology managed between January 2019 and January 2022. Epidemiological features, clinical aspects, and treatment modalities are presented. Results Six female patients and one male patient were operated; their age ranges from 11 to 67 years old. Etiologies are divided into infection, tumors, and pseudo tumors. Sphenoid sinus approach was performed through the sphenoethmoid recess in 5 cases, through the septum in one patient and through the ethmoid in one patient. Conclusion Endoscopic procedures have significantly improved the management of the sphenoid sinus pathology. They offer good access and control of the sphenoid pathology but require a perfect mastery of radiologic and endoscopic anatomy and its variations.