Mycobacterium abscessus complex (MABSC) is a rapidly growing mycobacterium and may rarely cause disseminated infections in immunocompromised patients. In patients with Cystic Fibrosis (CF), it peaks between the ages of 11 and 15 years. We present a 5 months old infant with coexisting CF and Progressive Familial Intrahepatic Cholestasis (PFIC) who had pulmonary and cutaneous dissemination of MABSC infection. The management of this disseminated infection in an infant with two coexisting chronic diseases was challenging which resulted in a rapid deterioration of lung disease and the progression of PFIC to liver cirrhosis with a fatal outcome. Keywords: Cystic Fibrosis; Atypical Mycobacterium; Mycobacterium abscessus complex; Progressive Familial Intrahepatic Cholestasis