Two patients with anti-centromere antibody (ACA), hypertensive emergency, and acute renal failure, mimicking scleroderma renal crisis, without Raynaud's phenomenon and typical skin manifestations of systemic sclerosis (SSc), are reported. A review of 26 ACA-positive patients between March 2003 and March 2011 in Yokosuka Kyosai Hospital identified four additional patients with similar manifestations. All patients were Japanese women between 41 and 84 years of age at presentation. Human leukocyte antigen (HLA) genotyping tests revealed the absence of the HLA-DQB1*0501 and DRB1*0101 alleles, which are associated with disease susceptibility to ACA-positive SSc among Japanese. These subjects' manifestations may represent a novel entity.