Crystalcryoglobulinemia refers to the extracellular deposition of crystals in the systemic vasculature, leading to vascular injury, thrombosis, and occlusion. This entity is usually described as a complication of multiple myeloma, although it can also occur in the setting of a monoclonal gammopathy of renal significance. Synchronous presentation of a monoclonal gammopathy and renal cell carcinoma is rare. Hypothetically, as both neoplasms’ growth depends on certain cytokines such as interleukin-6, it is possible that cytokine production from one neoplasm may stimulate the growth of the other. In this report, we describe a case of crystalcryoglobulinemia in a patient with a monoclonal gammopathy of renal significance and concomitant renal cell carcinoma. Additionally, we performed a systematic review of the literature and analyzed data of 61 reported cases of crystalcryoglobulinemia and of 40 reported cases describing the association between multiple myeloma and renal cell carcinoma.