Lymphomatous infiltration of the kidney is a rare manifestation of mantle cell lymphoma (MCL). We present the case of a 54-year-old male with MCL, acute kidney injury, extremely high antinuclear antibody titers, and hypocomplementemia. The diagnosis of MCL was further confirmed by kidney, lymph node, and bone marrow biopsies and immunohistochemical analysis of cyclin D1 expression. Renal ultrasonography demonstrated bilateral renal hypertrophy, and histological examination of renal biopsy revealed lymphomatous infiltration of the tubulointerstitium, with similar antigenic profiles to those observed in bone marrow and lymph node samples. Following a thorough literature search, we believe the present case represents the first report of MCL with abnormally high antinuclear antibody titers, hypocomplementemia, and acute kidney injury.