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Aim: to compare frequency and severity of neuro-ophthalmological symptoms in patients with different types of tumors based in the midbrain and pineal region. Background: Neuro-ophthalmological symptoms in patients with tumors of midbrain and pineal region were well described, but there are no studies which provide analysis of clinical manifestation of tumors depending on the pathomorphology of the neoplasm. Methods: This study included case series of 195 patients admitted to the Burdenko neurosurgical institute in 2005–2015. All patients were examined before biopsy or surgical removal. Further pathomorphological study revealed malignant tumors in 122 cases and benign ones in 73 cases. According to WHO among our patients there were 63 with tumor of the pineal region, 60 with germ cell tumor, 44 with astrocytic tumor, 15 with meningiomas and 13 with ependymomas. Results: We detected oculomotor and pupillary dysfunction in more than a half of the patients. Light near dissociation was the most frequent symptom. Midbrain disorders were observed in 87% of patients with malignant tumors and in 59% with benign ones. Papilledema was presented in 51% of patients with malignant tumors and in 34% of patients who had benign tumors. Comparing groups of patients with different types of tumors we revealed that oculomotor and pupillary disorders were more often in patients with germ cell tumors (93%) than in the other groups. Conclusion: frequency of clinical features in patients with midbrain and pineal region tumors depends on the pathomorphology of neoplasm and more common in patients with malignant tumors.