Neurological involvement may occur in 0.2%-52% of cases with trichinellosis, generally in the most severely affected patients. This review focuses on neurotrichinellosis and includes a brief overview of selected cases reported in the literature. Our primary goal was to increase the awareness of infectious diseases specialists, neurologists, and general practitioners about these major complications with possible fatal outcome. Seventy seven of the cases, for which enough details were available, have been pooled for statistical analysis. The mean age of the investigated group was 34.6 ± 16.8 years. Patients with both focal and diffuse manifestations predominated (55.8%), and they were significantly older (40 ± 15.5 years old) than those who presented solely focal (28.9 ± 17.8 years old; p = 0.03) or diffuse lesions (27.9 ± 15.3 years old; p = 0.007). In most of the cases (59.7%), complete recovery was reported, whereas 23.4% of cases had sequelae and 16.9% of the patients died. Patients who died had significantly lower eosinophil counts (13.8% ± 14%) when compared with those who made complete recovery (28.7% ± 18%; p = 0.015) and the cases with sequelae (35% ± 17.9%; p = 0.006). To sum up, trichinellosis must be considered in the differential diagnosis of any patient with encephalitis or other central nervous system malady of ambiguous etiology.