The clinical presentations of pulmonary embolism are varied and non-specific. It is often misdiagnosed or underdiagnosed in daily practice. Dyspnea, tachypnea and pleuritic chest pain are classic characteristics of pulmonary embolism. The diagnosis is facilitated when a patient with a risk factor presents with classic symptoms and signs. When a patient presents with atypical and limited symptoms or signs, the diagnosis of pulmonary embolism becomes even more challenging, especially if the clinical symptoms and signs suggest low or intermediate probability of this disease. We herein report on 4 cases of atypical presentation of pulmonary embolism. Clinicians should be alert to atypical presentations and include the diagnosis of pulmonary embolism on the basis of such findings. Our cases demonstrate the importance of pursuing the workup on patients with atypical presentations of pulmonary embolism, even if there is low clinical suspicion and nearly normal chest X-ray. These patients must be recognized to allow early and appropriate treatment that will lead to a good prognosis.