**Abstract**

This chapter describes the epidemiology, clinical and neuroimaging features, histological characteristics, surgical approach, outcomes, and prognostic factors of different cases of very rare intracranial tumors, associated with complex clinical syndromes. Highlighting the important aspects in the diagnosis and management that were considered relevant through the experience of our center. Here we included an intracranial Rosai-Dorfman disease manifested as an apparent multiple meningiomatosis, a choroid plexus papilloma clinically manifested as a hemifacial spasm originated by a compression of the facial colliculus, and a neuroenteric cyst associated with Klippel-Feil syndrome. This type of tumor presents a challenge to the neurosurgeon, originating various questions about its management. In this chapter, we present the experience we had with these pathologies to establish the most appropriate management decisions.

**Keywords:** rare intracranial tumors, multiple meningiomas, Rosai-Dorfman disease, Hemifacial spasm, choroid plexus papilloma, Klippel-Feil syndrome, neuroenteric cyst

## **1. Introduction**

Brain tumors according to their location and growth rate can produce very typical clinical manifestations [1], in addition to the classic characteristics of imaging studies that provide the possibility of approaching the diagnosis of the specific type of tumor and guide to establish the treatment modality [2]. However, when the incidence of some of these tumors is very low and they present with very varied clinical manifestations, added to the radiological findings that do not provide too much information to approximate the diagnosis, these cases condition stricter study protocols where the undoubtedly diagnoses alters the treatment modality for each particular case [3]. For this reason, knowledge of the existence of some of these tumors should be the subject of study, to understand the difficulty in diagnosis and treatment, seeking to reduce errors in addressing these cases and improve the result.
