RESUMEN Se describe el caso clinico de un paciente masculino de 17 anos de edad, que presento: talla baja, malformaciones oseo-esqueleticas, rasgos faciales toscos, hernia inguinal, hernia umbilical, disostosis multiples, hepatoesplenomegalia, y dificultad respiratoria; antecedentes quirurgicos: herniorrafia inguinal derecha y herniorrafia umbilical, alteraciones correspondientes a Mucopolisacaridosis (MPS) tipo VI, diagnostico confirmado por estudio inmunohistoquimico enzimatico, que reporto deficiencia de Arilsulfatasa. Se procedio al manejo farmacologico con Galsulfasa para controlar el progreso de los signos y sintomas caracteristicos, asi como las complicaciones producidas por la enfermedad. Palabras clave: Mucopolisacaridosis tipo VI, glicosaminoglicanos, disostosis multiples, Galsulfasa. MAROTEAUX-LAMY SYNDROME (MUCOPOLYSACCHARIDOSIS TYPE VI) ABSTRACT We describe the case of a male patient aged 17, who presented with short stature, bone-skeletal malformations, coarse facial features, inguinal hernia, umbilical hernia, multiple dysostosis, hepatosplenomegaly and difficulty in breathing; surgical history: right inguinal herniorrhaphy, umbilical herniorrhaphy, changes compatible with mucopolysaccharidosis (MPS) type VI. Diagnosis was confirmed by enzymatic immunohistochemical study, which reported arylsulfatase deficiency. Pharmacological management with Galsulfase was started, the progress of the characteristic signs, symptoms and complications caused by the disease were monitored afterwards. Key words: Mucopolysaccharidosis type VI, glycosaminoglycans, multiple dysostosis, Galsulfase.