Goat is considered as poor man’s cow and is among the earliest small ruminant species to be domesticated and are reared for meat and milk purpose, at least since 2500 B.C. in the Middle East. Goats contributes largely to the livelihood of livestock keeping households of low- and medium-input farmers, many of whom have few resources beyond their small holdings and livestock. It has been observed that maximum production can be obtained by protecting them from different prevalent diseases like enterotoxaemia. Enterotoxaemia is a fatal disease of small and large ruminant’s animal species; severe outbreak causes huge economic losses to livestock holders. This disease is also known as pulpy kidney disease due to systemic lesions observed on kidney as it causes nephritis and hydro nephrosis. Enterotoxaemia in goats occurs in four forms, i.e. Per acute, acute, subacute and chronic form. Severe outbreak of this disease observed in warm wet weather at the time of start of monsoon season every year and is having worldwide distribution. Factors which are responsible for exposing the animals towards the enterotoxaemia are sudden changing in diet as animals diet change from poor nutrient pastures to the rich nutrient pastures and changing in environmental temperature as warm wet weather is an ideal environment for the multiplication and toxins production of disease-causing microorganism. It can be diagnosed by detection of specific antibodies, clinical signs and post-mortem lesions. Infected animals can be treated by parental injection of specific chemotherapeutic agents or antitoxins and can be controlled by changing in dietary schedule and vaccination. Still, the pathology and pathogenesis of goat enterotoxaemia is not well understood, with limited studies available in goats. This review provides details information regarding the epidemiology, pathology and pathogenesis of enterotoxaemia in goat which might be helpful for future studies.