The hydatid disease, also referred to as hydatidosis or cystic echinoccosis (CE), is a parasitic zoonosis caused by the cestode parasite, Tinea echinococcus or tapeworm. It is a serious and potentially fatal disease that is still endemic in many parts of the world. Tinea echinococcus infection mainly affects the liver (cystic echinococcosis) caused by E. granulosus, and the lungs (alveolar echinococcosis, AE), associated with E. multilocularis/E. alveolaris, but has also been implicated in several unusual locations of the body. Carnivores such as dogs, wolves, and cats are the primary carriers and definitive hosts of the tapeworm, whereas herbivorous animals including sheep, goats, and cattle constitute the intermediate hosts, while human infections are often accidental. The illness in humans usually begins and proceeds asymptomatically and may become chronic thus manifesting later in life (≥10 years following infection), with attendant complications including anaphylactic shock from ruptured cyst. In most cases, the need for laboratory and other diagnosis, especially radiological (imaging) procedures, of hydatid disease is often based on high index of suspicion for the disease or the exigency for surgical operations prescribed for infected individuals. The use of serological tests such as enzyme-linked immunosorbent assay (ELISA) for the detection of parasite-specific IgG antibody in serum or plasma of patients has been recognized as a useful supplemental or confirmatory test to the clinical and radiological evaluations of hydatid disease. Despite poor standardization of laboratory procedures for diagnosis of hydatidosis, the use of purified antigens in serological diagnosis of echinococcosis has been found to improve precision and differentiation between E. granulosus (CE) and E. multilocularis (AE). However, the importance of these tests lies in their usage especially during the follow-up of treated patients following surgery, to detect disease resolution or recurrence so as to guide appropriate decision by the care givers. The use of polymerase chain reaction (PCR) is increasingly being recognized as a reliable diagnostic test for hydatid disease. Following increasing detection of hydatid cyst in areas considered to be extremely rare, its diagnostic tests are recommended in other organs (aside from liver and lungs) with high index of suspicion for the disease. Although diagnosis of hydatidosis is often based on radiologic imaging and serological tests, it is, however, being advocated that a definitive diagnosis using histopathological procedures should also be adopted during evaluation of the disease to improve precision of diagnosis to guide treatment.