Coccidioidomycosis was frequent in adult and paediatric populations in endemic areas of the United States, but it was observed in neonates on a few occasions. For a study, researchers sought to compare treated cases of neonatal coccidioidomycosis at a tertiary care children’s hospital in an endemic location to previously reported cases in the literature. From January 1, 2014, to December 31, 2019, they reviewed the medical records of infants aged one month or fewer hospitalised with coccidioidomycosis. In addition, they used PubMed to conduct a literature assessment of all known instances of newborn coccidioidomycosis throughout the previous 7 decades. The study did not include infants born to moms who had active coccidioidomycosis. At the facility, three cases of neonatal coccidioidomycosis were discovered. Each patient appeared differently and had a different diagnosis when they first arrived. On admission, 2 patients had negative coccidioidal screening tests but eventually seroconverted. All of the patients had extrapulmonary involvement; however, they all recovered after receiving the necessary treatment. According to a study of the literature, the symptoms and results of infant coccidioidomycosis vary greatly. Coccidioidomycosis could manifest in various ways in the newborn era, making diagnosis difficult. Coccidioidomycosis should be included in the differential diagnosis of ill-appearing infants who do not respond to treatment in endemic areas.