Poland syndrome is a rare congenital disorder typically characterized by unilateral absence or hypoplasia of the pectoralis major muscle, often accompanied by ipsilateral hand anomalies such as brachydactyly or syndactyly. While its clinical presentation is relatively well defined, associations with other congenital defects are uncommon. We report the case of an 18-year-old male who presented with left-sided chest wall asymmetry and brachydactyly, consistent with Poland syndrome, along with the novel finding of ipsilateral congenital ptosis—a feature not previously documented in the medical literature. Detailed physical, neurological, and ophthalmologic examinations, along with imaging studies, confirmed the diagnosis and ruled out other neurological etiologies. The absence of functional impairment led to conservative management with clinical monitoring. This case expands the known phenotypic spectrum of Poland syndrome and underscores the importance of thorough clinical evaluation in detecting atypical manifestations of rare congenital anomalies.