Acute appendicitis (AA) is one of the most frequent surgical emergencies, especially in pediatric populations, with its misdiagnosis in emergency settings presenting significant health risks. This misdiagnosis leads to various complications, such as delayed treatment or unnecessary surgeries. Factors such as age, gender, and comorbidities contribute to diagnostic errors, leading to complications such as peritonitis and increased negative appendectomy rates. This underscores the importance of accurate clinical assessment and awareness of common pitfalls, such as cognitive biases and over-reliance on laboratory tests. This review delves into the prevalence of AA misdiagnosis, its health burden, and the challenges inherent in the diagnostic process. It scrutinizes the effectiveness of different diagnostic approaches, including clinical assessment and imaging techniques. The treatment paradigms for AA are also explored, focusing on surgical interventions and the potential of conservative treatments using antibiotics. The review underscores the criticality of precise diagnosis in preventing adverse outcomes and ensuring effective treatment.