During the past 10 years, immunotactoid glomerulopathy has become recognized with increasing frequency. The lesion is characterized histologically by highly organized ultrastructural deposits that appear to be composed of immunoglobulin and complement and are negative for amyloid by Congo red stain. Clinically and/or serologically, patients have no evidence of cryoglobulinemia, amyloidosis, systemic lupus erythematosus, or a paraproteinemia, disorders associated with glomerular deposits, which also have a highly organized tactoidal or fibrillar characteristic. Immunotactoid glomerulopathy does not appear to be a multisystemic disease process and thus may represent a primary glomerulopathy. Patients with immunotactoid glomerulopathy present with proteinuria (nephrotic range in more than 60%) and over half of the patients have hypertension, hematuria, and renal insufficiency. Progression to end stage renal disease has occurred in more than 40% of patients reported to date. The experience in treating this disorder using prednisone and/or immunosuppression is limited and has not been impressive. Four patients have successfully undergone renal transplantation, but proteinuria recurred in two and was associated with the recurrence of immunotactoid glomerulopathy in the renal allograft. Although we have gained insight into the clinical course and histopathology of this disorder over the past few years, we still know little about its pathogenesis, an area for further research.