Mantle cell lymphoma (MCL) is a rare and aggressive subtype of non-Hodgkin lymphoma (NHL) originating from cells in the mantle zone. Herein, we present a case report of a 49-year-old male patient diagnosed with stage VI MCL, illustrating various diagnostic methods and procedures utilized in his evaluation. The patient presented with progressively enlarging cervical lymph nodes, fatigue, weight loss, anorexia, chronic diarrhea, abdominal pain, and rectal bleeding. Clinical examination revealed cutaneomucosal pallor, tachycardia, expiratory polypnea, bilateral jugulocarotid adenopathies, and splenomegaly. Laboratory investigations showed normocytic normochromic anemia, hyperleukocytosis, lymphocytic predominance, and thrombocytopenia. Peripheral blood smear analysis indicated a lymphoproliferative disorder, confirmed by flow cytometry revealing monotypic lymphoid cells. Bone marrow biopsy showed hypoplastic marrow with atypical cells. Immunohistochemistry confirmed mantle cell lymphoma localization. Subsequent thoraco-abdomino-pelvic CT scan revealed mediastinal and abdominal adenopathy with splenomegaly, leading to a diagnosis of stage IV MCL. The case highlights the importance of early and comprehensive diagnostic approaches in MCL management.