Neoplasms of striated and smooth muscle in children are a diverse group of neoplasms that have some unique aspects in contrast to these tumors in adults. Rhabdomyosarcoma is the most common soft tissue sarcoma of infancy and childhood and is relatively common in adolescents. In contrast, smooth muscle tumors are relatively rare, and the various types of rhabdomyoma and smooth and skeletal muscle hamartomas are very uncommon. In recent years, the understanding of the pathologic and genetic aspects of rhabdomyosarcoma has been enhanced by adjunct techniques, such as immunohistochemistry and cytogenetic or molecular genetic analysis. The current classification of rhabdomyosarcoma emphasizes the histologic-prognostic correlations. This article reviews the clinicopathologic features of striated and smooth muscle tumors with an emphasis on the unique aspects of these neoplasms in children and adolescents and the differential diagnosis.