Objective To study clinical and immunological features of renal tubular acidosis (RTA) patients complicated with autoimmunity disease. Methods Data of 60 patients of RTA complicated with autoimmune disease and 40 without it during 1999 and 2009 were reviewed, including clinical features,immunological examinations and renal tubular function. Results Among 60 patients of RTA, 59 were type Ⅰ, one type Ⅱ, and 55 complicated with Sjogen's syndrome (92%), one with systemic lupus erythematosus, nine with autoimmune thyroid disease, and one with rheumatoid arthritis. Flaccid paralysis was manifested in 50 (83%) RTA patients complicated with autoimmune disease, polyuria in 28 (47%),polydipsia in 28 (47%) , bone disorder in 24 (40%) and arthralgia in 28 (47%) cases, but of those without autoimmune disease, bone disease only in eight (20%) and arthralgia in nine (22%) cases, with statistically significant difference ( P ＜0. 01 ). Serum level of parathyroid hormone increased noticeably, with prevalence of positive antinuclear antibody, anti-SSA antibody and anti-SSB antibody of 88 % (49/56),84% (47/56) and 43% (24/56), respectively in RTA patients with autoimmune disease, all significantly higher than those in the patients without it ( P ＜ 0. 05 ). Conclusions Sjogen' s syndrome is the most common and prevalence of bone disorder and arthralgia are significantly higher in patients of RTA complicated with autoimmune disease, which should be examined as early as possible for timely diagnosis and treatment.