| Summary: | Abstract Sarcoidosis is an inflammatory disease known to be associated with multiple autoimmune disorders. There is a restricted number of descriptions of the association between sarcoidosis and autoimmune thrombotic thrombocytopenic purpura (aTTP). We present the case of a 63‐year‐old woman admitted to the hospital to investigate a possible sarcoidosis who had hemolytic anemia and thrombocytopenia, with low ADAMTS13 activity and anti‐ADAMTS13 antibodies, leading to a diagnosis of aTTP. Sarcoidosis was later confirmed and the two conditions evolved separately after 6 months, questioning the link between them. Clinicians should be aware of this rare cause of thrombocytopenia in patients with sarcoidosis, as aTTP is a life‐threatening condition.
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