Chronic Pulmonary Aspergillosis (CPA) is a destructive pulmonary disease caused by a fungal infection, affecting mainly individuals with prior or concurrent pulmonary conditions. It has a global prevalence of 42 per 100,000 population, but in the US and Europe, prevalence is less than 1 per 100,000. The clinical definition of CPA is based on various factors accounting for comorbidities, clinical presentation, and duration. It may be categorized into five subtypes that the disease may evolve between over time. Based on global consensus covering the spectrum of low-resource to high-resource settings, diagnosis is a multi-factorial process that involves a combination of clinical presentation persisting over 3 months, radiological findings, positive culture growth, and serological tests. CPA remains underdiagnosed due to a lack of awareness and is often misdiagnosed due to the comorbidities present. Treatment options are limited due to a lack of research. Furthermore, associated comorbidities and drug interactions further complicate treatment plans. Follow-up throughout treatment should be based on understanding the predictors of mortality. Identification of potential relapse or resistance to antifungal therapy is crucial to limit the low long-term survival rate. Awareness surrounding this devastating disease needs to be raised further to enable earlier identification, improve understanding of patient factors associated with prognosis, and the future potential for targeted therapies. This review aims to raise awareness of this rare condition among practitioners, by providing an overview of common risk factors influencing the prevalence and incidence of the disease. We further discuss current approaches and recent advancements in CPA diagnosis and treatment.