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Key Clinical Message Azathioprine, used for vasculitis and connective tissue diseases, carries long‐term cancer risks. This case report raises awareness among healthcare providers about such risks and emphasizes the need for taking necessary precautions to avoid them while treating such diseases. Abstract We present an Azathioprine‐induced lymphoma case in a 51‐year‐old male patient with Takayasu arteritis who presented with painless cervical swelling, itching, weight loss, and decreased appetite. This case report aims to increase awareness of the potential long‐term cancer risks associated with azathioprine use in the treatment of chronic diseases.