The metaphyseal dysostosis growth plate matrix was examined by alcian blue-magnesium chloride histochemistry and electron microscopy. The metaphyseal dysostosis matrix is uniformly alcian blue positive at all molarities of MgCl2 up to 1.0 M. In normal growth plates, the vertical septa become alcian blue negative at 0.5 to 0.6 M MgCl2. At 1.0 MgCl2, metaphyseal dysostosis plates are weakly periodic acid-Schiff (PAS) positive compared to intense PAS reactions in normal growth plates. The histochemical findings correlate well with the decreased collagen content of the matrix as seen by electron microscopy, resulting in a relative increase in proteoglycan granules. The findings indicate that the previously described cellular defect seen in metaphyseal dysostosis chondrocytes is related to an abnormal cartilage matrix that mineralizes poorly and is not easily degraded.