Pompe disease, is a rare severe, autosomal, recessive, and progressive genetic disorder caused by deficiency in alpha-glucosidase. The classic infantile-onset is the most broadly known form of Pompe disease, which presents with severe heart involvement and clear hypotonia, while the non-classic presentation occurs with early motor involvement. Late-onset Pompe disease develops in adults, but it may also occur during childhood or adolescence. We report a retrospective and descriptive study of a series of five Moroccan patients with confirmed diagnosis of IOPD; and we update the available clinical and diagnostic findings because an early management with enzyme replacement therapy may improve patients’ survival and quality of life.

Hydrops fetalis is defined by the accumulation of fluids in the serous membranes (pleurisy, ascites, pericarditis...), it is a rare condition; its etiologies are divided into immunological and non-immunological. Supraventricular tachycardia remains a known cause, non-immunological, but difficult to manage. In this article we report the case of a newborn with hydrops fetalis secondary to supraventricular tachycardia, which failed to be treated. The clinical, paraclinical, etiological diagnosis and finally the treatment were discussed.