RESUMO
We report a case of an 18-year-old male patient who was diagnosed with primary renal lymphoma (PRL) through biopsy findings and imaging studies. The patient presented with clinical manifestations of distal renal tubular acidosis including polyuria, polydipsia, lower limb weakness, involuntary weight loss, asthenia and dyspnea. No personal background or relevant medical history was reported. A kidney biopsy showed high grade immature B-cell lymphoproliferative process (Non-Hodgkin's Lymphoma) with a Ki67 value greater than 90%. Complementary studies excluded primary lymphoid migration sites, which confirmed the diagnosis of PRL. The oncology unit initiated treatment with a combination of medications due to lack of protocols for the specific treatment. Besides the fact that this condition is rare, it also shows a unique symptoms presentation and non-typical findings in imaging methods. Also, it is important to underline the fact that the treatment is not yet specified for such type of cancer and different combinations are needed to control the disease.