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The article presents a detailed analysis of clinical observation of primary diffuse large B-cell lymphoma of the adrenal glands, a rare disease affecting less than 1% of patients with non-Hodgkin’s lymphomas. It describes the case of a 46-year-old man hospitalized with progressive limb paralysis, later diagnosed as paraneoplastic polyradiculoneuropathy associated with bilateral adrenal lymphoma. The diagnostic challenges of this pathology, including ambiguous imaging results and the need for histological verification to confirm the diagnosis are discussed. Special attention is paid to the uniqueness of clinical manifestations and the importance of early symptom recognition to improve prognostic outcomes in highly aggressive forms of the disease. The necessity of multidisciplinary approach in managing such patients with the participation of oncologists, endocrinologists, and neurologists to optimize diagnostic and therapeutic processes is also emphasized.