Objective To study the clinical attributes, treatment and prognosis of acute promyelocytic leukaemia (APL) with translocation of t(11;17)(q23;q21). Methods A case of APL with t(11;17)(q23;q21) was reported, and the cytomorphology, immunology, cytogenetics and molecular genetics of the patient were analyzed. The related literature published in recent 20 years domestically and abroad was reviewed. Results The case herewith studied was a male patient aged 35 years with leucocyte count (WBC) of 38.17×109 in peripheral blood, and 88.5% of promyelocytes in bone marrow. Marrow karyotype analysis showed 46, XY, t(11;17)(q23;q21) and PLZF-RARA rearrangement was detected. The patient was definitely diagnosed as APL with t(11;17) (q23;q21). Complete morphological and genetic remission was achieved after a combined chemotherapy of As2O3 and ATRA, then a molecular biological remission was achieved after the treatment of 3 courses of large dose cytarabine. The patient was followed up to February 2013 with disease-free survival of nearly 10 months. Up to date, 20 cases of APL with t(11;17)(q23;q21) have been reported domestically and abroad, and their clinical attributes were reviewed. For the 20 cases, the mean age was 48.9±16.3 years and the male/female ratio was 9:1. Among the patients, males aged over 45 years accounted for 55%, implying that the elderly males may be high-risk population. The incidence of DIC was 60%, most of which got poor curative effect of ATRA. Conclusion APL with t(11;17)(q23;q21) is a very rare illness with distinct morphological changes and clinical characteristics. Prolonged combined chemotherapy with As2O3 and ATRA with large dose cytarabine may be beneficial to attain a remission and prolong survival.