Five cases of alveolar rhabdomyosarcoma with atypical clinical features are reported. Three patients showed lymphadenopathy as the first clinical manifestation, mimicking a lymphoma or a non identified primary tumor with lymph node metastases. One patient presented systemic neoplastic disease and two had the primary tumor in atypical locations, such as the mediastinum and retroperitoneum. All patients died and in four of them an autopsy was performed. The histological diagnosis was confirmed by immunohistochemical studies on four cases. The alveolar rhabdomyosarcoma has a poor prognosis and can have a variable clinical presentation and morphology, simulating lymphomas, leukemias and systemic metastatic disease with an unknown primary neoplasm, such as in the cases here in reported.