Due to its unspecific clinical presentation and the multitude of possible etiologies, chronic meningoencephalitis in immunosuppressed patients often represents a diagnostic challenge. Here, we report the clinical, radiological, cerebrospinal fluid, and microbiological findings of a 54-year-old male immunocompromised patient with mantle cell lymphoma and a 2-month history of brainstem and spinal meningoencephalitis. After unsuccessful treatment trials with antibiotics, a Candida albicans infection was confirmed by biopsy of a spinal cord lesion and large-volume cerebrospinal fluid culture. Therapy with liposomal amphotericin B/flucytosine and subsequent fluconazole resulted in significant clinical improvement. This case illustrates the importance of identifying the underlying cause of chronic meningoencephalitides in immunocompromised patients.