Caesarean scar pregnancy is one of the rarest forms of ectopic pregnancy. Little is known about its incidence and natural history. With increasing incidence of caesarean section worldwide, more and more cases are diagnosed and reported. Transvaginal ultrasound and colour flow Doppler provides a high diagnostic accuracy with very few false positives. A delay in diagnosis and/or treatment can lead to uterine rupture, major haemorrhage, hysterectomy and serious maternal morbidity. Early diagnosis can offer treatment options of avoiding uterine rupture and haemorrhage, thus preserving the uterus and future fertility. Management plan should be individually tailored. Available data suggest that termination of pregnancy is the treatment of choice in the first trimester soon after the diagnosis. Expectant treatment has a poor prognosis because of risk of rupture. There are no reliable scientific data on the risk of recurrence of the condition in future pregnancy, role of the interval between the previous caesarean delivery and occurrence of caesarean scar pregnancy, and effect of caesarean wound closure technique on caesarean scar pregnancy. In this article, we aim to find the demography, pathophysiology, clinical presentation, most appropriate methods of diagnosis and management, with their implications in clinical practice for this condition.