Clinical features in 10 cases (3 males, 7 females) with congenital muscular dystrophy (CMD) are reported. Six patients suffered from pure forms of CMD. Two patients had diffuse cerebral white matter involvement of both hemispheres, demonstrated by computerized tomography and magnetic resonance imaging. Two patients were diagnosed as having Walker-Warburg syndrome on the basis of clinical and neuroradiological findings. The present series of patients further illustrates the heterogeneity of the various entities included under the term CMD even when central nervous system involvement is not suspected.