A total of 137 patients with total colonic aganglionosis involving or not involving the small intestine (TCSA) were analyzed to clarify the recent trends of TCSA in Japan. The percent of TCSA out of all types of Hirschsprung's disease was 8.4%. Male to female ratio in TCSA was 1.5:1. Open biopsy was relatively frequent for diagnosis, the rate being 40.9%. A total of 67.4% of the TCSA patients were diagnosed in the first month of life. Seventy-two percent of the patients underwent enterostomy in the neonatal period. Definitive operations were performed in 67 patients. Sixty underwent retrorectal transanal pull-through, most of which were Martin's procedure (56 cases). Mortality rate after Martin's procedure was 12.5%. Sixty-four percent of the patients with enterostomy alone did not survive. This study shows the recent trends of TCSA and indicates there is still much room for improvement in the clinical management of TCSA.