Chagas disease (CD) is a vector-borne illness caused by the Trypanosoma cruzi parasite. It is the third most common parasitic infection worldwide and a major health problem in non-endemic regions. It can cause gastrointestinal and cardiac lesions due to cell infiltration and necrosis; common manifestations include cardiomyopathy and megaesophagus. Also, ischemic stroke is well documented, mainly due to cardioembolic etiology, although the correct management and etiological diagnosis is still challenging. Here we present a rare case of CD and ischemic stroke (IS) in a young patient, discussing the difficulties in correct diagnosis, treatment and gaps of knowledge in management and diagnosis of this neglected disease. It is important for stroke physicians to be aware of this condition, as a correct treatment, secondary prophylaxis and early referral to cardiology follow-up can potentially change drastically the natural history of the condition