An 89-year-old man presented with multiple cerebral infarction, and subsequently, thrombotic thrombocytopenic purpura (TTP) was diagnosed. He was treated with plasma exchange (PEX), however, despite the initial response, he was refractory to PEX. The addition of rituximab led to a sustained complete response. This case presents the oldest known reported TTP patient treated with rituximab and shows that rituximab can be administered successfully to very elderly patients with refractory TTP, and that therapeutic modalities similar to those for younger patients can be considered for this age group. This case also shows that elderly TTP patients may present with uncommon symptoms.