Multinodular and vacuolating neuronal tumor (MVNT) have been recently added to the WHO classification of CNS tumors and has not been extensively reported upon in the radiological literature. We report the first radiological and the largest series of cases, aiming to highlight the natural history of lesions with the imaging appearance of MVNT with long follow-up time. In this retrospective study, we collected cases with the imaging appearance of MVNT. All lesions were evaluated by using routine MR imaging, with follow-up of up to 93months. Patient demographics, clinical course, and MRI features of the lesions were recorded. Twenty-four subjects were enrolled, f/m=16:8, age range 24-59years, with a median age of 45years. The patients' symptoms were often episodic and most frequently due to headaches in 12 (50%), visual symptoms in 6 (25%), seizures in 5±1 (20-25%), paresthesia in 4 (~17%), cognitive difficulties in 4 (~17%), in addition to other variable neurological symptoms, or incidental. A total of 30 lesions identified, 77% of the lesions had gadolinium-enhanced MRI and only 13% showed enhancement. A 6.7% of the lesions that had MRI followed up showed progression, while the rest remained stable up to 93months interval. All patients had intact neurological examinations (except one case that was diagnosed with optic neuritis), were managed conservatively, and did well. The natural history of lesions with imaging features of MVNT is overall stable from a clinical and imaging appearance over time.