This document reports a case of atypical Bovine Spongiform Encephalopathy (BSE), type H, detected in Brazil. The case involved an 11-year-old beef cow from a farm in Mato Grosso that was submitted for emergency slaughter. Testing at the national reference laboratory confirmed the presence of prion antigens. This is the fifth case of atypical BSE identified in Brazil since surveillance began 23 years ago. Control measures were implemented, including removal and destruction of specified risk materials. The investigation of this isolated event has concluded.