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Extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) is most commonly found in the GI tract. Other less common anatomical sites for MALT include the skin, intestine, salivary glands, lungs, and ocular adnexa. Isolated MALT of the kidney has only been sporadically reported. Most of the reported cases in the literature present with underlying renal mass and are generally diagnosed post nephrectomy. We present a case of a 73-year-old gentleman with biopsy-proven primary MALT of the kidney who presented with a
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