Objective: Optochiasmatic cavernoma is an extremely rare cerebral lesion. They account for approximately 1% of all cavernomas of the central nervous system. Reports on this pathology are limited. Abrupt visual deterioration is a common symptom of the disease. Treatment strategy and visual outcomes after different treatment approaches remain a subject for discussion. Methods: Patients operated in a period 2005-2021 were analyzed in this study. All patients preoperatively underwent computed tomography (CT) scan, CT-angiography, and magnetic resonance imaging (MRI). Visual function of the patients was assessed pre-op, post-op and at the follow-up. Duration of visual dysfunction was noted as well. Surgical details were also extracted from medical notes. All patients were followed up, and control MRI was performed one month after operation. We assessed surgical series of optochiasmatic cavernomas published for last 10 years. Further comparative analysis with our data was performed. Results: Five patients were included into this study. There were four men and one woman. Mean age comprised 33.8 years (range 20-48 years). Most patients were admitted to our hospital due to visual disturbances (80%). Visual function improved in four patients. Visual function was unchanged in one patient, lacking visual disturbancies pre-op. Complication developed in one patient. Conclusions Optochiasmatic cavernomas are encountered extremely rare. Despite the use of contemporary diagnostic options, differential diagnosis remains challenging. Full diagnostic work-up is mandatory. After the diagnosis is made, surgical treatment should be considered first. Total microsurgical or endoscopic transsphenoidal removal of the optochiasmatic cavernoma is a relatively safe and effective treatment method facilitating improvement of visual function.